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PostScript ..............................................................................................
Wegener’s granulomatosis
Wegener’s granulomatosis (WG) is a systemic
granulomatous inflammatory disease of un-
known origin. It occurs at any age, with the
peak incidence in the third and fourth
decades. The classic diagnostic triad of WG is
necrotising granuloma of the upper or lower
respiratory tract, vasculitis, and nephritis.
Identifying a raised ANCA titre is virtually
diagnostic of the disease, especially with the
classic granular cytoplasmic staining pattern
of neutrophils (C-ANCA).1 This disease is
usually fatal if untreated; however, early diag-
nosis and instigation of immunosuppressants
such as cyclophosphamide can induce long
term remission.2
Case report
A 79 year old woman presented with a 2 week
history of a painful, red left eye. She had mild
arthritis and was also noted to be profoundly
deaf, requiring hand written notes for com-
munication. There was no history of sinus
problems.
Her best corrected visual acuities were 6/18
in the right eye and 6/12 in the left eye. The
left peripheral supratemporal cornea showed
three areas of 80% corneal thinning, 1 × 1 mm
in size, with an overlying epithelial defect (Fig
1). There was adjacent conjunctival injection
and moderate anterior chamber cellular activ-
ity. The other eye was normal with no signs of
inflammation. Examination of her hands
showed no obvious rheumatoid abnormali-
ties. Initial investigations performed were full
blood count, electrolytes, C reactive protein,
erythrocyte sedimentation rate (ESR), and
rheumatoid factor. Corneal scraping and
cultures were also undertaken to exclude an
infective cause. The only significant test result
was a raised ESR of 94, with other tests
including creatinine being within the normal
range. A provisional diagnosis was made of
rheumatoid corneal melt and treatment was
commenced with topical dexamethasone
0.1% non-preserved hourly and oral pred-
nisolone at 40 mg/day.
After 5 days of steroid treatment, the
corneal thinning was resolving and the
conjunctival injection settling (Fig 2). Also by
the fifth day, the patient’s hearing had
dramatically improved. She could now con-
duct a conversation at normal volume. On
further questioning, she said her hearing had
deteriorated only over the past few months
and had ascribed it to “natural” old age dete-
rioration. This suggested a link between her
Figure 1 Peripheral corneal melt with
adjacent conjunctival injection.
www.bjophthalmol.com
Figure 2 Resolving peripheral corneal melt
and conjunctival injection.
corneal changes and hearing loss and she was
investigated for Wegener’s granulomatosis
(WG). Her serum antineutrophil cytoplasmic
antibodies (cANCA) titre was found to be
raised (>160). Rheumatoid factor was nega-
tive.
Oral cyclophosphamide was commenced on
diagnosis of WG and the oral prednisolone
slowly tapered. Her ocular inflammation,
hearing, and arthritis continued to improve
on treatment and at the 2 month follow up,
there was minimal peripheral corneal thin-
ning and no inflammation. Best corrected
visual acuity in the left eye remained at 6/12.
Examination by an otolaryngologist found
mild hearing impairment and no significant
sinus pathology. Rheumatological review
found only mild arthropathy.
Comment
The majority of the ocular involvement in WG
is caused by focal vasculitis, involving the
anterior and/or posterior segment of the eye
and corneal melting has been reported in
11–16% of patients with WG.3 4 Profound sen-
sorineural deafness is a less common feature,
occurring in about 6% of patients on
presentation.2 5 It occurs either secondary to
inflammation of the cochlear vessels or serous
otitis media.4 Other important systemic fea-
tures include pyrexia, weight loss, recurrent
epistaxis, sinus discharge, haemoptysis, per-
ipheral neuropathy, cerebral vasculitis, and
renal failure (major cause of death). Fortu-
nately in this case, the patient had the limited
form of WG, with no renal involvement.
This patient presented with an uncommon
combination of corneal melt and profound
deafness. As deafness is a relatively common
problem among the elderly patient
population6 it may be overlooked as an impor-
tant symptom, especially if ocular pathology is
the main feature at presentation. It was not
until the patient’s hearing improved on treat-
ment with oral prednisolone that its signifi-
cance became apparent. WG is a rare condi-
tion and early recognition and treatment are
essential as the disease carries a significant
risk of serious complications. A full systemic
history in patients with corneal melt is
important in formulating the correct diagno-
sis and thereby expediting the appropriate
treatment.
K S Lim, GA Lee, C E Pavesio, L A Ficker
Moorfields Eye Hospital, London, UK
Br J Ophthalmol 2003;87:500–519
Correspondence to: Graham A Lee, Queensland,
Australia; mdglee@hotmail.com
Accepted for publication 12 August 2002
References
1 Van der Woude FJ. Anticytoplasmic
antibodies in Wegener’s granulomatosis.
Lancet 1985;2:48.
2 Fauci AS, Haynes BF, Katz P, et al.
Wegener’s granulomatosis: prospective
clinical and therapeutic experience with 85
patients for 21 years. Ann Intern Med
1983;98:76–85.
3 Haynes BF, Fishman ML, Fauci AS, et al. The
ocular manifestations of Wegener’s
granulomatosis. Fifteen years’ experience and
review of the literature. Am J Med
1977;63:131–41.
4 Bullen CL, Liesegang TJ, MacDonald TJ, et al.
Ocular complications of Wegener’s
granulomatosis. Ophthalmology
1983;90:279–90.
5 Thornton MA, O’Sullivan TJ. Otological
Wegener’s granulomatosis: a diagnostic
dilemma. Clin Otolaryngol 2000;25:433–4.
6 Davis AC. The prevalence of hearing
impairment and reported hearing disability
among adults in Great Britain. Int J Epidemiol
1989;18:911–17.
Pigmentary retinopathy, macular
oedema, and abnormal ERG
with mitotane treatment
Adrenocortical carcinoma is a rare tumour
with a poor prognosis. Mitotane (o,p’-DDD), a
chemotherapy drug that suppresses the adre-
nal cortex and modifies peripheral steroid
metabolism has been reported to cause ocular
side effects including visual blurring, diplopia,
cataract, toxic retinopathy with retinal haem-
orrhage, oedema, and papilloedema. We
present a 32 year old woman with reduced
visual acuity, retinal pigmentation, macular
oedema, and abnormal ERG after taking
mitotane. While primary hypoadrenalism in
Addison’s disease has never been reported to
cause any retinal problem, secondary hypoad-
renalism in adrenoleucodystrophy is associ-
ated with pigmentary retinopathy and other
ocular findings. We postulate that the retinal
problems secondary to mitotane treatment
may act via a similar mechanism.
Case report
A 30 year old woman had a left nephrectomy,
adrenalectomy, and chemotherapy in Septem-
ber 1997 following diagnosis of an adrenal
carcinoma. In 1999, she was found to have
secondary tumours in her lungs and liver. She
was commenced on intra-arterial cisplatin
and oral mitotane of up to 4.5 g daily for 6–8
months, ceasing in December 1999 because of
weight loss, malaise and, soon after that,
marked decrease of visual acuity in both eyes.
The patient had no family history of any reti-
nal disease. She had worn glasses for myopia
for 9 years with best corrected visual acuity of
6/4 each eye previously.
On 2 March 2000 her visual acuity was 6/12
in the right eye and 6/60 in the left eye. She
also had facial pigmentation. Funduscopy
showed extensive pigmentary clumping in
each eye and macular oedema in the left side
(Fig 1). She was commenced on cortisone
acetate and fludrocortisone to attempt to
improve her vision.
PostScript
Figure 1 Right fundus showing mottled
pigmentation at the macula.
One week later her visual acuity was 6/18 in
the right and 6/24 in the left. Fundus
examination showed mottled pigmentation at
the macula and mid-periphery of both eyes
but macular oedema in her left eye had
resolved. Disc and vessels were normal. Full
field electroretinography (ERG) was per-
formed. Her rod response (scotopic, dim blue
stimulus) b-wave was 50% of normal, the
cone response (photopic, red stimulus) had
b-wave 75% of normal amplitude and delayed
implicit time (Fig 2). The amplitude of 30 Hz
flicker responses was also reduced. There was
no significant asymmetry between the two
eyes. On 30 March 2000, her visual acuity
improved to 6/12 in the right and 6/9 in the
left. Her facial pigmentation had also faded.
Comment
Surgical resection is the treatment of choice
for adrenocortical carcinoma.1 Mitotane (o,p’-
DDD) is the only drug that causes regression
of metastases and improves survival.2 3 Its
biochemical action is unknown but data sug-
gest that it modifies peripheral steroid me-
tabolism and directly suppresses the adrenal
cortex. The incidence of ocular side effects
was 4% in a study of 132 patients.3 These
effects include visual blurring, diplopia, lens
opacities, optic neuritis, and a toxic retin-
opathy with features of papilloedema and
retinal haemorrhage. In another study involv-
ing 19 patients, three patients had toxic retin-
opathy that included papilloedema, small
retinal haemorrhages, and oedema and an-
other patient had a subcapsular cataract.4 To
Figure 2 Full field ERG.
our knowledge neither pigmentary retin-
opathy nor abnormal ERG findings have been
reported previously. Previous studies also did
not mention side effects reversibility except
for a case of lenticular opacities that disap-
peared 5 days after discontinuance of
mitotane.3 In our case, the patient’s visual
acuity did improve significantly, with drying
out of macular oedema after cessation of
therapy and initiation of steroid replacement.
It is possible, however, that the ocular
changes in this patient were not caused by
mitotane but were secondary to cancer
associated retinopathy (CAR). Its characteris-
tic findings include attenuated retinal arteri-
oles, with limited, if any, clinically apparent
retinal pigmentary changes and cells in the
vitreous humour.5 CAR was considered to be
unlikely in this case because of the normal
calibre of the retinal arterioles, the absence of
vitreous cells, and the timing of the onset.
Patients with CAR experience visual symp-
toms that often precede or are concurrent
with the tumour diagnosis.6
Although primary hypoadrenalism in Addi-
son’s disease has never been reported to cause
retinal problems, secondary hypoadrenalism
in adrenoleucodystrophy is associated with
pigmentary retinopathy and other ocular
findings. Adrenoleucodystrophy is a group of
rare lipid storage disorders with increased
serum level of long chain fatty acids (C24–
C30). Ocular findings in adrenoleucodystro-
phy include visual loss secondary to visual
tract demyelination and primary retinal gan-
glion cell degeneration, squint, cataracts, loss
of corneal sensation, abnormal visual evoked
potentials, and macular pigmentary changes.7
The retinal pigmentary changes observed his-
tologically were different from those in retini-
tis pigmentosa.8
We postulate that the retinal side effects of
mitotane could occur via a similar mechanism
that affects the metabolism of long chain fatty
acids. Mitotane causes a reduction in plasma
17-hydroxy corticosteroids level but an in-
crease in the levels of 6-beta-hydroxyl cortisol,
cholesterol, liver enzymes, corticosteroid
binding globulin, and sex hormone binding
globulin.1 At the time of writing, there was no
published information on whether mitotane
treatment affects serum long chain fatty acid
levels. The availability of such data will be
valuable for evaluating this postulation.
501
W T Ng, M G Toohey, L Mulhall,
D A Mackey
St Vincent’s Hospital, Fitzroy, Victoria, The Ballarat
Eye Clinic, Ballarat, Victoria, Ocular Diagnostic
Clinic and CERA, Royal Victorian Eye and Ear
Hospital, East Melbourne, Victoria, Australia
Correspondence to: David Mackey;
d.mackey@utas.edu.au
Accepted for publication 12 August 2002
References
1 Luton J-P, Cerdas S, Billaud L, et al. Clinical
features of adrenocortical carcinoma,
prognostic factors and the effect of mitotane
therapy. N Engl J Med 1990;322:1195–201.
2 Lubitz JA, Freeman L, Okun R. Mitotane use
in inoperable adrenal cortical carcinoma.
JAMA 1973;223:1109–12.
3 Hutter AM, Kayhoe DE. Adrenal cortical
carcinoma, results of treatment with o,p’-DDD
in 138 patients. Am J Med 1966;41:581–92.
4 Hoffman DL, Mattox VR. Treatment of
adrenocortical carcinoma with o,p’-DDD. Med
Clin North Am 1972;56:999–1012.
5 Jacobson DM, Thirkill CE, Tipping SJ. A
clinical triad to diagnose paraneoplastic
retinopathy. Ann Neurol 1990;28:162–67
6 Fishman GA, Birch DG, Holder GE, et al.
Electrophysiologic testing in disorders of the
retina, optic nerve and visual pathway. 2nd
ed. San Francisco: The Foundation of the
American Academy of Ophthalmology,
2001:118–120.
7 Traboulsi EI, Maumenee IH. Ophthalmologic
manifestations of X-linked childhood
adrenoleukodystrophy. Ophthalmology
1987;94:47–52.
8 Glasgow BJ, Brown HH, Hannah JB, et al.
Ocular pathologic findings in neonatal
adrenoleukodystrophy. Ophthalmology
1987;94:1054–60.
Coxsackievirus B4 associated
uveoretinitis in an adult
Coxsackievirus is a kind of enterovirus, which
may cause respiratory and gastrointestinal
symptoms, erythema, meningoencephalitis,
myocarditis, pericarditis, and myositis. Al-
though Coxsackievirus B4 is apparently a rare
cause of chorioretinitis, Coxsackievirus infec-
tion and concurrent posterior segment in-
flammation have been disclosed in a few
cases. However, iridocyclitis, scattered haem-
orrhagic dots, and occlusive retinal vasculitis
have not previously reported as being features
of Coxsackievirus B4 infectious ocular dis-
ease.
Case report
A 34 year old man was referred to our hospi-
tal with a 2 week history of inferior visual
field defect and visual loss in his right eye. His
left eye was asymptomatic. A few days before
the onset of visual manifestations, the patient
noted a prodrome of viral infection, consisting
of severe headache and high grade fever of
38.0–39.2°C, joint pain, and general malaise.
There was no significant medical or family
history. At the initial ophthalmic examina-
tion, his best corrected visual acuities were
10/20 in the right eye and 25/20 in the left eye,
and intraocular pressure was normal in both
eyes. Slit lamp examination showed moderate
cellular infiltration in the anterior chamber in
both eyes. Humphrey central visual field test-
ing demonstrated relative scotoma inferior to
fixation in the right eye. Goldmann peripheral
visual field testing was within normal limits
in both eyes. Ophthalmoscopic examination
disclosed subretinal exudates with haemor-
rhage in the parafovea and the mid-periphery
of bilateral eyes (Fig 1). Moreover, scattered
www.bjophthalmol.com
502
Figure 1 Photographs of right eye (A) and left eye (B) at the initial ophthalmic examination.
Subretinal exudates with haemorrhage around the macula and near the disc, and scattered
haemorrhagic blots are evident.
Figure 2 Fluorescein angiograms of the
right eye. Note blocked fluorescence and
slight dye leakage from the retinal vessels
around the macula, and irregular choroidal
background fluorescence.
haemorrhagic dots and blots were observed
along retinal vessels. The optic discs appeared
normal. Fluorescein angiography demon-
strated occlusive retinal vasculitis character-
ised by blocked fluorescence and mild dye
leakage from the retinal vessels correspond-
ing to the lesions detected clinically, and
irregular choroidal background fluorescence
(Fig 2). Laboratory examination findings were
normal except a white blood cell count of
10 500 ×106/l, GOT of 76 IU/l, GPT of 209 IU/l,
gamma-GTP of 88 IU/l, and LDH of 771 IU/l.
Serological tests, however, revealed a slight
elevation of serum antibody to Coxsackievirus
B4, at a titre of 1:8 (neutralisation test).
The patient’s general condition improved
gradually without specific medication. Cellu-
lar infiltration in the anterior chamber gradu-
ally disappeared. The retinal exudative lesions
reduced in size, and haemorrhage tended to
decrease. Eight months after the initial
ophthalmic examination, bilateral exudative
lesions had improved and were replaced by
scars, and most of the retinal haemorrhagic
lesions had disappeared. Visual acuity was
restored to 25/20 in the right eye and 30/20 in
the left eye. However, relative paracentral
scotoma persisted in his right eye. Antibody
titre to Coxsakievirus B4 had increased, dem-
onstrating a 16-fold rise between the acute
(1:8) and convalescent sera (1:128). Retro-
spectively, we diagnosed the ocular lesions in
this patient as uveoretinitis induced by
Coxsackievirus B4 infection.
Comment
Viruses are one of the most common causes of
infections involving the posterior segment of
the eye. Such infections may be congenital or
www.bjophthalmol.com
acquired, and may affect primarily the retina
or the choroid. Retinitis and choroiditis
caused by viruses such as measles, influenza,
Epstein-Barr virus, and Rift Valley fever virus
typically occur on an acquired basis, subse-
quent to an acute viral systemic illness.
Coxsakievirus associated chorioretinitis is
thought to elicit the same clinical course as in
other viral chorioretinitis. Coxsackievirus in-
fection and concurrent posterior segment
inflammation have been reported in a few
cases, in which clinical signs and antibody
titres suggested that Coxsackievirus was the
responsible agent.1–4 Among them, two cases
of chorioretinits associated with Coxsackie-
virus B4 infection were reported.1 3 One was a
paediatric case, and the ophthalmoscopic
manifestation was described as scattered
white lesions in mid-periphery of the retina.1
The other was an adult case, with chori-
oretinitis similar to the multiple evanescent
white dot syndrome.3 However, the clinical
features observed in our patient consisted of
iridocyclitis, scattered haemorrhagic dots, and
occlusive retinal vasculitis were apparently
different from the two earlier reports.
Coxsackievirus infection is apparently a
rare cause of uveoretinitis but, nevertheless,
should be considered in the appropriate clini-
cal setting, since it is possible that overt clini-
cal manifestations and complications may
occur depending on the severity and the loca-
tion of the uveoretinal lesions.
M Takeuchi, J Sakai, M Usui
Tokyo Medical University, 6-7-1 Nishishinjuku
Shinjuku-Ku, Tokyo, Japan
Correspondence to: Masaru Takeuchi;
takeuchi@tokyo-med.ac.jp
Accepted for publication 30 August 2002
References
1 Hirakata K, Oshima T, Azuma N.
Chorioretinitis induced by coxsackievirus B4
infection. Am J Ophthalmol
1990;109:225–7.
2 Forster W, Bialasiewicz AA, Busse H.
Coxsackievirus B3-associated panuveitis. Br J
Ophthalmol 1993;77:182–3.
3 Kadrmas EF, Buzney SM. Coxsackievirus B4
as a cause of adult chorioretinitis. Am J
Ophthalmol 1999;127:347–9.
4 Haamann P, Kessel L, Larsen M. Monofocal
outer retinitis associated with hand, foot, and
mouth disease caused by coxsackievirus. Am J
Ophthalmol 2000;129:552–3.
Evaluation of telemedicine for
slit lamp examination of the eye
following cataract surgery
Ophthalmic surgery is well suited to the use
of telemedicine in diagnosis and
PostScript
management,1 2 but diagnostic accuracy and
reliability are critical if it is to be widely
introduced.3 Moorfields Eye Hospital is lo-
cated in central London and runs community
outreach clinics. One clinic and operating list
in Ealing is 17 miles from the central hospital.
The surgical teams operate in Ealing and then
return to the central hospital. We therefore
investigated the use of a telemedicine link for
review of postoperative cataract surgical pa-
tients aiming to assess what can be seen
clearly and reliably using telemedicine and to
identify what the observer using this link may
potentially miss.
Case report
This study had Moorfields Eye Hospital
research and ethics committee approval.
Patients consecutively admitted for cataract
surgery were recruited to the study and full
informed written consent was obtained. A
telelink of a pair of Global Telemed mobile
workstations connected by three ISDN lines,
with a video transmission rate of 384 bit/s was
used for examinations,1 with high resolution
examination being achieved by static images.
Two surgeons conducted examinations, one
using the slit lamp and the other using the
telemedicine link. Slit lamp signs graded by
the two observers comprised the presence and
degree of the following—central corneal
oedema, corneal oedema at the limbal section,
Siedel’s sign, folds in Descemet’s membrane,
anterior chamber depth, flare and cells,
intraocular lens decentration, and lens stabil-
ity. Both observers in person independently
examined a group of postoperative cataract
patients in order to determine agreement
between practitioners.
Twelve consecutive postoperative cataract
patients were recruited to the study with 10
others as a control group to assess interob-
server agreement by in-person examination.
The results of the study are given in Table 1.
Comment
Although interobserver variation means that
telelink reliability cannot be precisely calcu-
lated in terms of sensitivity and specificity,
these findings give an indication of the accu-
racy of slit lamp examination after cataract
surgery using 384 K bandwidth. Examination
by video telelink was relatively reliable in
detecting oedema at the central cornea but
did not consistently detect oedema at the cor-
neal section, or anterior chamber flare and
failed entirely to detect DM folds or anterior
chamber cells. Patients enjoyed the telemedi-
cine experience finding it reassuring to see as
well as interact with their surgeon via the tel-
elink.
Video compression algorithms used in
streaming video are “lossy” and higher band-
width increases video quality. The process
begins with a good video source with low
noise, since noise does not compress well. A
poor quality video image is very difficult to
stream successfully because of this difficulty
in compression. The videoconferencing stand-
ard H-261 was implemented to provide for
video compression to a given ISDN band-
width. With increasing bandwidth there is
correspondingly better quality video. A tele-
video link for corneal assessment in Canada
using six ISDN lines (768 K) was found to be
acceptable, whereas three lines (384 K) were
unacceptable (personal communication, Dr M
Pop, Montreal, Canada).
The video display also affects the infor-
mation observed. Broadcast video monitors
are accurately colour calibrated, whereas dig-
ital compressed video to 384 K bandwidth has
PostScript
Table 1 Consistency of scoring by the two observers
Oedema of the incision χ2=2.933, p=0.087
Oedema of the central cornea
Descemet’s folds
Anterior chamber cells
Anterior chamber flare
Lens implant stability
Management decisions
reduced colour depth.4 Recognition of corneal
oedema is less about well defined structure as
subtle display of colour and “haze.”
It is felt by the authors that telemedicine is
a wholly different experience from a simple
telephone conversation and for many situa-
tions is capable of facilitating postoperative
assessment with trained operators. H-261
video at 384 K (triple ISDN) is sufficient for
anterior segment overall examination but is
not sufficient for detailed corneal assessment.
Other practitioners have used data rates up to
four times faster for more detailed
examination.5
L F F Smith, J Bainbridge, J Burns,
J Stevens, P Taylor, I Murdoch
Moorfields Eye Hospital, City Road,
London ECIV 2PD, UK
Correspondence to: Lindsey Smith;
101506.1271@compuserve.com
Accepted for publication 2 September 2002
References
1 Murdoch I, Bainbridge J, Taylor P, et al.
Postoperative evaluation of patients following
ophthalmic surgery. J Telemed Telecare
2000;6(S1):84–6.
2 DeSutter E, DeMolder R, Gabirel P.
Tele-ophthalmology. The TIME project: a
tele-medicine project in the region Nord-Pas
de Calais and Zuid-West Vlaanderen. Bull Soc
Belge Ophtalmol 1994;252:37–42.
3 Li HK. Telemedicine and ophthalmology. Surv
Ophthalmol 1999;44:61–72.
4 Solari SJ. Digital video and audio
compression. London: McGraw-Hill, 1997.
5 Threlkeld AB, Fahd T, Camp M, et al.
Telemedical evaluation of ocular adnexa and
anterior segment. Am J Ophthalmol
1999;127:464–6.
Inherited retinal dystrophy and
asymmetric axial length
The prenatal and postnatal development of
the eye is determined by complex interactions
between a number of genes, their products,
and certain environmental factors.1 2 Since
each eye is influenced by precisely the same
processes as its fellow, mutations in regula-
tory genes usually lead to symmetric pheno-
types. In this report, we describe two siblings
of Asian ethnicity, born to unrelated parents
with no family history of ocular disease, who
have an unusual bilateral retinal dystrophy
associated with very asymmetrical ocular
growth.
Case report
Sib 1 was an 8 year old boy with epilepsy who
had been born at term after an uneventful
pregnancy. Pendular nystagmus had been
noted soon after birth and his visual acuity in
each eye was 3/60; refraction −17.00 DS right,
plano left. Anterior segment examination was
Control group Study group
(n=10) (n=12)
7/10 (70%) 4/12 (33%)
7/10 (70%) 10/12 (83%)
10/10 (100%) 7/12 (58%)
9/10 (90%) 0/12 (0%)
9/10 (90%) 6/12 (50%)
9/10 (90%) 12/12 (100%)
9/10 (90%) 11/12 (93%)
unremarkable with normal intraocular pres-
sures. Examination of the right eye (Fig 1A
and B) revealed generalised retinal pigment
epithelial and choroidal atrophy, a macula
“coloboma,” sheathed and occluded retinal
vessels inferotemporally, and a shallow infe-
rior longstanding retinal detachment with
subretinal fibrosis. The left eye appeared simi-
lar; however there was no macula abnormal-
ity. By ultrasound, the axial length and ocular
volume were 26.70 mm and 9.9 ml right, 20.70
mm and 4.6 ml left respectively.
Sib 2 was a 6 year old girl born at term with
no significant past medical history. Her
corrected visual acuity was 3/60 in each eye;
refraction −17.00 DS right, plano left. Anterior
segment examination was normal. Fundus-
copy of the right eye revealed a “macula colo-
boma,” extensive retinal pigment epithelial
and choroidal atrophy, intraretinal pigment
migration and preretinal fibrosis. The appear-
ances of the left eye were similar. No retinal
detachment was noted. Axial lengths and
ocular volumes were 26.1 mm and 9.4 ml
right, 20.7 mm and 4.6 ml left, respectively.
Electrophysiology was performed on both
children based on the ISCEV standards for
adults, but initially using surface electrodes
on the lower eyelids. No definite ERG re-
sponse could be recorded from the emme-
tropic left eye of either child (even with high
intensity stimulation or photopic flicker
stimulation with averaging), a finding con-
sistent with severe generalised dysfunction
involving rod and cone photoreceptors. The
electrical responses of both children’s myopic
fellow eye showed a milder degree of dysfunc-
tion: there were reduced b:a ratios in both
scotopic and photopic responses which, to-
gether with the abnormal 30 Hz flicker ERGs,
suggest a mid-retinal locus affecting post-
phototransductional cone and rod systems.3
The skin recordings obtained with surface
Figure 1 Colour photographs of right fundus of sib 1 (A) showing macular “coloboma,”
generalised retinal pigment epithelial and choroidal atrophy, and evidence of sheathed and
occluded retinal vessels inferotemporally; (B) same eye, shallow inferior retinal detachment
with subretinal fibrosis.
503
electrodes were confirmed under anaesthesia
using gold foil corneal electrodes.
It is reasonable to conclude that these two
siblings represent original probands with a
novel inherited, probably autosomal recessive,
retinal dystrophy. The particularly interesting
feature of this disorder is the asymmetry of
the axial lengths and ocular volumes of the
eyes, rare in genetically determined ocular
diseases. Indeed, we are aware of only one
other report that identifies ocular asymmetry
in association with retinal degeneration.
Lafaut et al4 reported a single patient who had
bilateral Stargardt’s disease and unilateral
myopia.
Normally, axial elongation and ocular en-
largement are carefully coordinated to equal-
ise growth of fellow eyes in the quest for
emmetropia.1 The differences observed in the
two siblings described here reflect a decou-
pling of this developmental synchrony. It has
been demonstrated that form deprivation in
various species produces progressive axial
growth and myopia of the postnatal eye.5
However, in both our sibs the ERG responses
were much better preserved in the highly
myopic right eye where, in addition, there was
a low b:a ratio. Similar findings do not occur
in myopia without retinal pathology.6
The bilateral symmetrical retinal dystrophy
noted in both our patients suggests a genetic
basis for the disease but a single genetic defect
cannot explain the additional asymmetry of
eye size and electrophysiological measure-
ments. Most probably, the phenotype results
from at least two separate events, a germline
mutation in a retinal or retinal pigment
epithelial specific gene leading to degenera-
tion, thereby creating a susceptible back-
ground on which a second event could occur.
This event, leading to the asymmetry of ocular
volume, may be an environmental factor or a
mutation in a second gene important in the
regulation of eye growth. If this were a
somatic rather than germline mutation it
would explain the asymmetry of the disease.
P Francis, A G Robson, G Holder,
A Moore
Moorfields Eye Hospital, City Road, London
EC1V 2PD, UK
P Francis, A Moore
Institute of Ophthalmology, University College
London, London EC1V 9EL, UK
S Kaushal
University of Minnesota, Department of
Ophthalmology and Institute of Human Genetics,
9-337 Phillips-Wangensteen Building, 516
Delaware Street SE, Minneapolis, MN 55455, USA
www.bjophthalmol.com
504 PostScript

Correspondence to: Professor A T Moore,

Department of Molecular Genetics, Institute of
Ophthalmology, University College London 11–43
Bath Street, London EC1V 9EL, UK;
tony.moore@ucl.ac.uk

Accepted for publication 3 September 2002

References
1 Brown N, Koretz J, Bron A. The development
and maintenance of emmetropia. Eye
1999;13:83–92.
2 Crewther D. The role of photoreceptors in the
control of refractive state. Prog Retina Eye Res
2000;19:421–57.
3 Fishman G, Birch D, Holder G, et al.
Electrophysiologic testing in disorders of the
retina, optic nerve and visual pathway. San
Francisco: The Foundation of the American
Academy of Ophthalmology, 2001.
4 Lafaut B, van Egmond J, de Laey J.
Asymmetric fundus flavimaculatus/Stargardt’s
disease, associated with unilateral myopia. Int
Ophthalmol 1995–6;19:253–5.
5 Wildsoet C, Schmid K. Optical correction of
form deprivation myopia inhibits refractive
recovery in chick eyes with intact or sectioned
optic nerves. Vis Res 2000;40:3273–82.
6 Perlman I, Meyer E, Haim T, et al. Retinal
function in high refractive error assessed
electroretinographically. Br J Ophthalmol
1984;68:79–84.

Adenoma of ciliary pigment

epithelium: a case series
Adenoma of ciliary pigment epithelium is a
rare tumour. Many are diagnosed retrospec-
tively either after excision or enucleation, as
malignant melanoma is suspected.1 We report
a series of four patients found to have
adenoma of ciliary pigment epithelium and
discuss the clinical features and unusual
behaviour of these neoplasms.

Case reports
We reviewed the histopathological reports in
the ophthalmic pathology archive dating from
1980 to date and identified four patients who
had the histopathological diagnosis of ad-
enoma of ciliary pigment epithelium. We
crosschecked the details with the clinical
oncology database. We reviewed their notes
for features that would help us to identify this
ciliary body tumour clinically. The salient fea-
tures of these patients are given in Table 1.
Patient 1 was reported elsewhere in 1994.2
He had a dark brown multinodular mass in
the inferotemporal anterior chamber angle of
the left eye. His tumour was a relatively small
but invasive lesion. Patient 2 was the only
non-white patient with this condition in our
series. Her tumour was an incidental finding
when she presented to an ophthalmologist
with allergic conjunctivitis. The tumour was
small and dark brown. The tumour had
invaded the anterior chamber angle and the
root of the iris occupying one clock hour of the
angle (Fig 1A, B, and C). Adenoma of the cili-
ary body was suspected, as she was non-white
Figure 1 (A) A small ciliary body adenoma invading the angle in patient 2. (B) Gonioscopic
view of the ciliary body adenoma (A) that had invaded the angle and the root of the iris, also
showing the localised cataract through the pupil. (C) Ultrasound biomicroscopy of the
adenoma of ciliary body shown in (A) and (B). (D) Pigment clumps in the vitreous in patient 3.
(E) Shows the adenoma of the ciliary body, the dark iris, and the trabeculectomy site in patient
4.
and the degree of anterior chamber invasion Patient 4 was initially treated for acute
appeared disproportionate to the size of the angle closure glaucoma in another hospital.
tumour. Trabeculectomy was performed to achieve
Floaters and blurred vision were the pre- control of intraocular pressure. Postopera-
senting symptoms in patient 3. The vitreous tively, he was found to have a lesion behind
showed presence of pigment clumps and the the crystalline lens. He underwent phaco-
extensive pigment dispersion made the media emulsification with intraocular lens implanta-
hazy (Fig 1D). The tumour was a solid dark tion to improve visualisation of the lesion. A
brown lesion arising from the ciliary body black ciliary body mass was seen (Fig 1E).
between the 12 and 1 o’clock meridians. The This prompted his referral to the oncology
clinical differential diagnosis was between service in June 1999. Control of the intraocu-
malignant melanoma and adenocarcinoma of lar pressure proved refractory even with addi-
the pigment epithelium of ciliary body. tional medical treatment.

Table 1 Clinical details of the patients with adenoma of the pigment epithelium of ciliary body
Year of
No Age/sex/race VA Size (mm) Clinical features Surgery Recurrence diagnosis Complication

1 40/M/W 6/5 8×7×1 Angle invasion Local resection No 1992 RD repair

2 62/F/A 6/6 5×3×1 Angle invasion, cataract Local resection No 1996
3 65/F/W 6/18 8×6×4 Sentinel vessel, pigment dispersion Local resection No 1998 RD repair
4 54/M/W 1/60 5×4×4 Secondary glaucoma, pigment dispersion Enucleation No 1999

M = male, F = female, W = white, A = Asian, RD = retinal detachment, VA = visual acuity.

www.bjophthalmol.com
PostScript
Pigment dispersion was seen in patients 3
and 4. This was mainly in the vitreous of
patient 3 but in both the vitreous and the
anterior segment of patient 4. There was het-
erochromia of the iris in patient 4. No angle
invasion was seen in these two patients.
Other associated features were a localised
cataract in patient 2, tractional retinal detach-
ment and secondary glaucoma in patient 4.
Patient 3 had an episcleral sentinel vessel over
the tumour. None of these patients had any
history of ocular trauma or intraocular in-
flammation. Ultrasound biomicroscopy (Fig
1C) helped us to evaluate these tumours in
more detail.
The first three patients underwent local
resection of the tumour in the form of
iridocyclectomy under hypotensive anaesthe-
sia. The last patient had enucleation as he
opted to have the eye removed because of the
poor visual prognosis for that eye as a result of
secondary glaucoma, extensive pigment dis-
persion, and tractional retinal detachment.
Histopathologically, these tumours showed
heavy pigmentation. Mitotic activity was
absent or low. Invasion of ciliary muscle and
the iris root was seen in patients 1 and 2.
Patient 3 had a cystic adenoma with cells
forming gland-like structures around central
cysts.
Comment
Our series highlights the paradoxical behav-
iour of adenoma of ciliary pigment epithe-
lium. Smaller lesions invaded the angle and
larger lesions caused extensive pigment dis-
persion although non-invasively. Angle inva-
sion resulted in these tumours being seen and
resulted in the presentation of patients 1 and
2. Blurred vision due to pigment dispersion in
the vitreous resulted in the presentation of
patient 3. Angle closure glaucoma and pig-
ment dispersion were the main features of
patient 4. Shields et al1 presented a series of
eight patients with adenoma of ciliary pig-
ment epithelium and described their clinical
features. In their series they found an associ-
ation with cataract, vitreous haemorrhage,
and neovascular glaucoma.
Pigment dispersion was seen in two of our
patients. The presence of pigment clumps and
extensive pigment dispersion in the vitreous
of patient 3 (Fig 1D) is unique and has not
been reported before. Chang et al3 in 1979
reported the presence of pigment in the retro-
lental space adjacent to the tumour in a case
of adenoma of ciliary pigment epithelium.
Extensive pigment dispersion in vitreous had
been reported in malignant melanoma of the
choroid4 but to our knowledge not in ad-
enoma of ciliary pigment epithelium.
Secondary glaucoma from intraocular tu-
mours is well known. In their survey of
intraocular tumours causing secondary glau-
coma Shields et al5 reported on 2704 eyes. Of
the five adenomas of the ciliary body one was
from ciliary pigment epithelium. None of
these had secondary glaucoma. Of the ciliary
body melanomas, 17% had secondary glau-
coma. Angle closure was responsible for
secondary glaucoma in 12% of the eyes with
ciliary body melanoma. In their series in 1999
Shields et al1 had one patient who had neovas-
cular glaucoma secondary to adenoma of cili-
ary pigment epithelium. Patient 4 in our series
presented with secondary angle closure glau-
coma.
Malignant melanoma of ciliary body is
known to invade the anterior chamber angle.
Chang et al3 reported angle invasion in
adenoma of the pigment epithelium of ciliary
body. Shields et al6 reported a patient in whom
invasion of the iris stroma by an adenoma of
ciliary pigment epithelium was documented
with progressive growth. They initially sus-
pected this to be a tumour of the iris but on
later evaluation showed the origin from the
ciliary body. The presenting feature in patients
1 and 2 in our series was similar, although the
ciliary body origin was recognised initially. In
patient 2 the diagnosis of adenoma of ciliary
body was strongly suspected preoperatively, as
the tumour that had invaded the angle was
very small. Invasion of the angle by ciliary
body melanomas usually does not occur until
they have attained a larger size. Invasion of
the angle has also been described in melano-
cytoma of the ciliary body.7 They too tend to be
relatively larger when they invade the angle,
unlike the adenomas that we described. Iris
melanocytomas undergo central necrosis and
cause pigment dispersion and glaucoma.8
However the necrotic centre is absent in
adenomas.
One of our patients (patient 1) had a senti-
nel vessel. Sentinel vessels are typically
thought to be associated with malignancy.
However, this is not always the case. Presence
of a sentinel vessel indicates ciliary body
involvement. Fine needle aspiration biopsy
may be considered for aiding diagnosis of
malignancy. However, its role in the diagnosis
of these lesions may be limited. Absence of
malignant cells does not always rule out the
presence of malignancy.
Our study highlights the paradoxical be-
haviour of adenoma of the pigment epithe-
lium of ciliary body that has not been empha-
sised before. Adenoma of the pigment
epithelium of the ciliary body should be kept
in mind if there is extensive pigment disper-
sion by larger tumours and invasion of the
anterior chamber angle by relatively small
tumours.
S Dinakaran, P A Rundle
Department of Ophthalmology, Royal Hallamshire
Hospital, Glossop Road, Sheffield S10 2JF, UK
M A Parsons
Ophthalmic Sciences Unit
I G Rennie
Department of Ophthalmology and Orthoptics,
University of Sheffield
Correspondence to: Mr S Dinakaran;
sdinakaran@yahoo.com
Accepted for publication 4 September 2002
References
1 Shields JA, Shields CL, Gunduz K, et al.
Adenoma of the ciliary body pigment
epithelium: the 1998 Albert Ruedemann, Sr,
memorial lecture, part 1. Arch Ophthalmol
1999;117:592–7.
2 Rennie IG, Faulkner MK, Parsons MA.
Adenoma of the pigmented ciliary epithelium.
Br J Ophthalmol 1994;78:484–5.
3 Chang M, Shields JA, Wachtel DL. Adenoma
of the pigment epithelium of the ciliary body
simulating a malignant melanoma. Am J
Ophthalmol 1979;88:40–4.
4 El Baba F, Hagler WS, De la Cruz A, et al.
Choroidal melanoma with pigment dispersion
in vitreous and melanomalytic glaucoma.
Ophthalmology 1988;95:370–7.
5 Shields CL, Shields JA, Shields MB, et al.
Prevalence and mechanisms of secondary
intraocular pressure elevation in eyes with
intraocular tumours. Ophthalmology
1987;94:839–46.
6 Shields JA, Eagle RC, Shields CL, et al.
Progressive growth of benign adenoma of the
pigment epithelium of the ciliary body. Arch
Ophthalmol 2001;119:1859–61.
7 LoRusso FJ, Boniuk M, Font RL.
Melanocytoma (magnocelluler nevus) of the
505
ciliary body: Report of 10 cases and review of
literature. Ophthalmology 2000;107:795–
800.
8 Fineman MS, Eagle RC, Shields JA, et al.
Melanocytomalytic glaucoma in eyes with
necrotic iris melanocytoma. Ophthalmology
1998;105:492–6.
Phenylephrine 2.5% and 10% in
phacoemulsification under
topical anaesthesia: is there an
effect on systemic blood
pressure?
Phenylephrine 10% leads to a faster and more
pronounced mydriasis but cardiovascular ef-
fects like hypertension and arrhythmias have
been reported. In a young healthy adult the
upper limit of safety for intravenous adminis-
tration of phenylephrine is 1.5 mg1 and
Kumar et al2 had found phenylephrine plasma
levels after administration of topical 10% vis-
cous solution to their patients to be 1.842–
11.526 ng/ml after 20 minutes while topical
2.5% aqueous solution produced plasma levels
of 0–1.720 ng/ml after 20 minutes. They
concluded that the mean pressure tended to
be higher with the 10% viscous solution.
Chin et al,3 in their study on 89 patients,
concluded that significant hypertensive ef-
fects can arise after topical phenylephrine;
however, no significant difference between
the two groups (2.5% v 10%) was shown.
Symons et al4 reported no significant change
in the mean systolic and diastolic blood pres-
sure in 126 patients receiving 10% phenyl-
ephrine. Malhotra et al5 in their study on 54
cases showed no difference in systemic
cardiovascular effects of either the 2.5% or the
10% concentration.
Methods
We carried out a prospective randomised and
double masked study on 53 patients undergo-
ing phacoemulsification under topical anaes-
thesia (Amethocaine 0.1% and Marcaine
0.75%) and no exclusion of cases with hyper-
tension, heart failure, or diabetes was made.
All patients received one drop of each of tropi-
camide 1%, cyclopentolate 1%, and Voltarol
0.1%. Phenylephrine drops, either 2.5% or
10%, were administered in three doses start-
ing half hour before surgery at 5 minute
intervals. All patients had their blood pressure
measured before dilatation and in the anaes-
thetic room preoperatively then postopera-
tively.
Results
The study included 49 cases aged 52–91, 24
cases were known to have cardiovascular
problems and seven were also diabetic. Com-
parison of the blood pressure changes before
Figure 1 Blood pressure (mm Hg) with
phenylephrine 2.5%. Numbers 1 and 2 =
systolic and diastolic reading preanaesthetic;
3 and 4 = systolic and diastolic reading in
the anaesthetic room; 5 and 6 = systolic and
diastolic reading in recovery.
www.bjophthalmol.com
506
Table 1 t Test 2.5% and 10% (normotesives and hypertensives)
Mean
Normotensive/sys −12.44
Normotensive/diast 2.88
Hypertensive/sys 14.16
Hypertensive/diast −4.16
Two tailed t test, comparing the 2.5% group and 10% group both hypertensive and normotensive
patients.
*p=0.05.
dilatation and in the anaesthetic room preop-
eratively in the 2.5% group (Fig 1 showed a
mean rise in systolic BP of 22 mm Hg
(p=0.003) in the normotensive group. In the
hypertensive group all patients recorded
statistically significant rises in systolic and
diastolic BP (respectively p=0.010, p=0.009).
In the 10% group (Fig 2, 10 patients in the
normotensive group showed a mean rise in
systolic BP of 34.4 mm Hg (p=0.001) and 10.5
mm Hg in diastolic BP (p=0.077). In the
hypertensive group six patients recorded a
rise in systolic BP with a mean of 22.8 mm Hg
(p=0.015) and a diastolic rise with a mean of
16.8 mm Hg (p=0.033) (Table 1) (Figs 3 and
4).
Comment
Our study showed that patients in both
groups of phenylephrine doses experienced a
rise in systolic blood pressure which was sta-
tistically significant; the mean of which is
higher in the 10% group. The maximal systo-
lic blood pressure rise is around 10–20
minutes after administration, which corre-
sponds to the time of maximal plasma levels
of phenylephrine as demonstrated by other
authors,5 but the paired t test failed to show
any significance between the results of the
groups of either concentration. Postoperative
results showed some elevations in systolic and
diastolic BP but weren’t statistically signifi-
cant. Using Altman’s normogram the study
was limited in its power. Although the aim of
this study was not designed to investigate the
effect of anxiety on systemic blood pressure,
this element should be taken into considera-
tion since it is known to elevate the systolic
blood pressure. We have also used topical
anaesthesia so that adrenaline 1:200 000,
which is always included in local anaesthesia,
is excluded which might cause elevation of
the blood pressure although the concentra-
tion is too low for any significant systemic
effect.
Figure 2 Blood pressure (mm Hg) with
phenylephrine 10%. Numbers 1 and 2 =
systolic and diastolic reading preanaesthetic;
3 and 4 = systolic and diastolic reading in
the anaesthetic room; 5 and 6 = systolic and
diastolic reading in recovery.
www.bjophthalmol.com
SD Significance*
19.08 0.86
16.64 0.61
28.36 0.27
14.00 0.49
Figure 3 Blood pressure (mm Hg) with
phenylephrine 2.5% The mean changes to
systolic and diastolic BP.
Figure 4 Blood pressure (mm Hg) with
phenylephrine 10% The mean changes to
systolic and diastolic BP.
We therefore recommend the routine use of
the 2.5% phenylephrine in ophthalmic sur-
gery and only use 10% solution for cases
where the lower concentration may not be as
effective.
N B Kenawy, M Jabir
Department of Ophthalmology, Rotherham General
Hospital, Moorgate Road, Oakwood, Rotherham
S60 2UD, UK
Correspondence to: Dr N Kenawy;
nkenawy@yahoo.co.uk
Accepted for publication 4 September 2002
References
1 Fraunfelder FT, Scafidi AF. Possible adverse
effects from topical ocular 10%
phenylephrine. Am J Ophthalmol
1978;85:447–53.
2 Kumar V, Schoenwald RD, Chien DS, et al.
Systemic absorption and cardiovascular effets
of phenylephrine eye drops. Am J Ophthalmol
1985;99:180–4.
3 Chin KW, Law NM, Chin MK. Phenylephrine
drops in ophthalmic surgery: a clinical study
on cardiovascular effects. Med J Malaysia
1994;49:158–63.
4 Symons RCA, Walland MJ, Kaufman DV.
Letter to the editor. Eye 1997;11:946–7.
5 Malhotra R, Banerjee G, Brampton W, et al.
Comparison of the cardiovascular effects of
2.5% phenylephrine and 10% phenylephrine
during ophthalmic surgery. Eye
1998;12:973–5.
PostScript
Effect of preoperative detection
of photoreceptor displacement
on postoperative foveal findings
in eyes with idiopathic macular
hole
In idiopathic macular hole formation, Gass1
hypothesised that macular holes enlarge with
displacement of the photoreceptors without
tissue loss around the fovea. Jensen and
Larsen2 developed binocular kinetic perimetry
that could evaluate local retinal photoreceptor
displacement and also confirm centrifugal
photoreceptor displacement away from the
foveola in eyes with a macular hole. Using the
binocular perimetry technique and scanning
laser ophthalmoscope microperimetry, we
confirmed that the patients with a unilateral
macular hole with preoperative photoreceptor
displacement had better postoperative visual
function when compared to patients without
preoperative photoreceptor displacement.3 We
also previously reported that the postopera-
tive foveal findings were correlated with the
postoperative visual function in eyes with
macular hole.4
The purpose of the present study was to
investigate whether or not detecting photore-
ceptor displacement preoperatively affects the
postoperative foveal findings in eyes with an
idiopathic macular hole.
Methods and results
Fifteen patients (12 women, three men; mean
age 62 (SD 5) years) with a unilateral
idiopathic macular hole that resolved after
vitreous surgery were examined. All had
undergone surgery at our institution. Eleven
eyes were classified as having a stage 3 hole,
four stage 2, and one stage 4. The best
corrected visual acuity (VA) levels in the
unaffected eyes were 0.7 or better, and no
patients had ocular diseases except for mild
cataract.
To detect photoreceptor displacement in
eyes with the disease preoperatively, we
performed binocular kinetic perimetry.2 3 The
anatomical status of the repaired macular
holes was assessed 3 months after the opera-
tion using optical coherence tomographic
equipment (OCT 2000, Humphry Instrument,
division of Carl Zeiss, San Leandro, CA, USA).
Good postoperative foveal findings were de-
fined as the presence of a foveal depression, as
previously reported by Imai and associates5
(Figs 1 and 2).
Photoreceptor displacement was detected
in 11 of the 15 (73%) patients preoperatively.
Good postoperative foveal findings were ob-
served in nine of the 11 (82%). Photoreceptor
displacement was detected preoperatively in
all nine patients. The prevalence of good post-
operative foveal findings was significantly
higher in patients in whom photoreceptor
Figure 1 Good postoperative foveal
findings observed with optical coherence
tomography. A foveal depression is
observed.
PostScript
Figure 2 Bad postoperative foveal findings
observed with optical coherence
tomography. A foveal depression is not
observed.
displacement (82%, 9/11) was detected preop-
eratively than in those in whom displacement
was not detected (0%, 0/4) (p=0.01, χ2 test
with Yates’s correction). The mean preopera-
tive VA tended to be better and the mean VA
measured 3 months postoperatively was sig-
nificantly better in patients in whom photore-
ceptor displacement was detected preopera-
tively (0.15 and 0.79, respectively) than in
those whom it was not detected (0.08 and
0.25, respectively) (p = 0.06 and p = 0.001,
respectively, unpaired t test). Visual acuities
were converted to the logarithm of the
minimum angle of resolution (logMAR) for
statistical analysis.
Comment
Jensen and Larsen2 reported that in two
macular hole patients with the longest dura-
tion of symptoms, photoreceptor displace-
ment was not detected. The reason why
patients with a macular hole with longer
duration of symptoms did not have photore-
ceptor displacement might be that there was
more damage to the displaced photoreceptors.
A longer disease duration probably induced
functional damage to the displaced photore-
ceptors, and consequently no photoreceptors
are displaced from the fovea. We also reported
that the detection of photoreceptor displace-
ment preoperatively should affect postsurgical
visual function, and photoreceptor damage
might occur in eyes in which photoreceptor
displacement was not detected preoperatively,
resulting in worse postoperative visual
function.3
Our results demonstrated that in patients
with a macular hole in whom photoreceptor
displacement was detected preoperatively, the
postoperative foveal findings and visual acui-
ties were better when compared to patients in
whom displacement was not detected preop-
eratively. It was also reported that better ana-
tomical foveal recovery in eyes after macular
hole surgery resulted in better improvement
of postoperative visual function.4 Although
limitations of this study are based in its sam-
ple size, we think that a degree of preoperative
photoreceptor damage influences not only
postoperative visual function but also ana-
tomical recovery.
The authors have no proprietary interest in any
instruments used in this study.
T Hikichi, N Kitaya, S Konno,
J Takahashi, F Mori, A Yoshida
Department of Ophthalmology, Asahikawa Medical
College, Asahikawa, Japan
Correspondence to: Taiichi Hikichi, MD,
Department of Ophthalmology, Asahikawa Medical
College, 2-1 Midorigaoka-higashi, Asahikawa
078-8510, Japan; hikichi@asahikawa-med.ac.jp
Accepted for publication 5 September 2002
References
1 Gass JDM. Reappraisal of biomicroscopic
classification of stages of development of a
macular hole. Am J Ophthalmol
1995;119:752–9.
2 Jensen OM, Larsen M. Objective assessment
of photoreceptor displacement and
metamorphopsia. a study of macular holes.
Arch Ophthalmol 1998;116:1303–6.
3 Hikichi T, Kitaya N, Takahashi J, et al.
Association of preoperative photoreceptor
displacement and improved central scotoma
after idiopathic macular hole surgery.
Ophthalmology (in press).
4 Hikichi T, Ishiko S, Takamiya A, et al.
Scanning laser ophthalmoscope correlations
with biomicroscopic findings and foveal
function after macular hole closure. Arch
Ophthalmol 2000;118:193–7.
5 Imai M, Iijima H, Gotoh T, Tsukahara S.
Optical coherence tomography of successfully
repaired idiopathic macular holes. Am J
Ophthalmol 1999;128:621–7.
Nodular scleritis in a patient
with sarcoidosis
Sarcoidosis is an immune mediated disease
that may affect any organ. Scleral involvement
has rarely been described with sarcoidosis.1–3
We report on a patient with unilateral nodular
anterior scleritis who was found to suffer
from sarcoidosis. Here, the clinical and his-
topathological features are described.
Case report
A 53 year old white woman had persistent
moderate tenderness in her left eye that lasted
for several weeks. Visual acuity was 20/20 in
both eyes. The slit lamp appearance of the
right eye was unremarkable. A scleral nodule
in the inferior nasal quadrant was found in
the left eye (Fig 1), and a moderate tenderness
was determined in this area. The intraocular
pressures were within normal ranges. On
ophthalmoscopy, the posterior segment of
both eyes was normal. The ultrasound images
did not indicate posterior scleritis.
The medical history was remarkable for an
episode of hepatitis A 20 years ago, and the
patient suffered from exercise dependent dys-
pnoea, night sweats, and repeated headaches.
An extensive examination for associated
systemic diseases was performed, including
serological tests for syphilis, CRP, ESR, ANCA,
RF, CIC, ANA, and PPD tests: all were within
normal ranges and consultant examinations
by the internal medicine, rheumatology, neu-
rology, and ENT departments were performed.
The chest x ray examination revealed a
Figure 1 Scleral nodule of the left eye with
tenderness in a patient with sarcoidosis.
507
Figure 2 Histopathology of a scleral
nodule in a patient with sarcoidosis. The
scleral tissue with oedema and vessel
dilatation, perivascular lymphocytic cell
infiltration with non-caseating granuloma-like
cell accumulation, some histiocytes and giant
cells. Haematoxylin and eosin staining,
magnification, ×200.
bilateral hilar lymphadenopathy and basal
interstitial pulmonary infiltration typical of
sarcoidosis stage 2. In the body plethysmo-
graphy, a mild restriction and a moderately
reduced diffusion capacity was determined.
The bronchoalveolar lavage revealed an in-
creased proportion of CD3+ T cells and an
increased CD4/CD8 ratio, consistent with
active pulmonary sarcoidosis. The level of
angiotensin converting enzyme was 51.3 U/l
(normal, 8–52) in the high normal range. The
magnetic resonance images revealed an in-
creased enhancement and thickening of the
inferior nasal part of the sclera of the left eye.
A biopsy was taken from the scleral nodule
under peribulbar anaesthesia. Histopathologi-
cal studies on the paraffin embedded tissue
that was performed by the pathologists
revealed a perivascular lymphocytic cell infil-
tration, with non-caseating granuloma-like
cell accumulation (Fig 2). Some histiocytes
and very few giant cells were found. The scle-
ral tissue was oedematous and the vessels
were dilated, but necrosis, vasculitis or malig-
nant cell configurations were absent. Addi-
tionally, Ziehl-Neelson and Gonori’s silver
methaminamine stains excluded the possi-
bility of causative infections.
Treatment with oral prednisone with an
initial dosage of 1 mg/kg was performed. The
corticosteroid dosage was tapered within 6
weeks, and was maintained at 20 mg. Under
this regimen, the pain and the scleral nodule
resolved immediately, and the ACE level fell to
24 U/l (normal).
Comment
Scleral involvement has been previously re-
ported in a few patients with sarcoidosis. In a
series of 537 patients with histologically
proved sarcoidosis,3 scleral plaques anterior to
the equator have been found in four cases, and
this was seen in the acute stage of disease and
in association with erythema nodosum and
bihilar lymphadenopathy. In another series by
Jabs and Johns,2 within a group of 183
patients with chronic sarcoidosis, scleral
involvement was detected in one patient.
However, the clinical course and histopatho-
logical appearances have not been described
previously. Posterior scleritis has been seen
only rarely with sarcoidosis.1
In our patient, the sparse systemic symp-
toms made the diagnosis difficult and, there-
fore, scleral biopsy was done in order to rule
out infections or malignancies and to disclose
the histopathological evidence for sarcoidosis.
Although a classic non-caseating granuloma
www.bjophthalmol.com
508
has not been found in our patient, a
granuloma-like perivascular cell infiltration
was seen, consisting primarily of lym-
phocytes, some histiocytes, and very few giant
cells, which are typical for sarcoidosis. To the
best of our knowledge, this is the first
histopathological description of a sarcoid
lesion in the sclera. The mainstay of treatment
for sarcoidosis is corticosteroids, and the scle-
ritis responded in our patient.
Acknowledgements
The authors thank Professor Dr Annette Fisseler-
Eckhof, Department of Pathology, Zentralklinik Emil
von Behring in Berlin and Professor Dr Christian
Witting, Department of Pathology, Clemens Hospi-
tal, Muenster for the histopathological evaluation of
the tissue specimens.
A Heiligenhaus, D Michel, J M Koch
Department of Ophthalmology, St Franziskus
Hospital, Muenster, Germany
Correspondence to: Arnd Heiligenhaus, MD,
Department of Ophthalmology, St Franziskus
Hospital, Hohenzollernring 74, 48145 Muenster,
Germany; arnd.heiligenhaus@t-online.de
Accepted for publication 5 September 2002
References
1 Dodds EM, Lowder CY, Barnhorst DA, et al.
Posterior scleritis with annular ciliochoroidal
detachment. Am J Ophthalmol
1995;120:677–9.
2 Jabs DA, Johns CJ. Ocular involvement in
chronic sarcoidosis. Am J Ophthalmol
1986;102:297–301.
3 James DG, Neville E, Langley DA. Ocular
sarcoidosis. Trans Ophthalmol Soc UK
1976;96:133–9.
Panophthalmitis following
contact diode laser
cyclophotocoagulation in a
patient with failed
trabeculectomy and
trabeculotomy for congenital
glaucoma
Diode laser trans-scleral cyclophotocoagula-
tion (TSCPC) is a well accepted method for
the management of patients with refractory
glaucoma. A lasting decrease in IOP has been
reported in about two thirds of eyes with
severe, medically uncontrolled glaucoma fol-
lowing contact TSCPC using G-probe delivery,
with preservation of visual acuity.1 2 Although
the procedure is considered safe,3 4 complica-
tions have been reported.1 5 Here, we report
the first case, to the best of our knowledge, of
panophthalmitis following contact diode laser
cyclophotocoagulation using the G-probe in a
patient with failed trabeculectomy and
trabeculotomy for congenital glaucoma.
Case report
An 18 year old male patient presented to our
emergency service with a 4 day history of
pain, redness, and periorbital swelling in the
right eye, accompanied by poor vision. One
week earlier he had undergone 360 degree
diode laser trans-scleral cyclophotocoagula-
tion using contact G-probe in the right eye for
uncontrolled glaucoma. He had thrice under-
gone filtering surgeries in both eyes for
congenital glaucoma since 1985. During the
last trabeculectomy performed in 1996, intra-
operative mitomycin C (0.2 mg/100 ml applied
for 3 minutes beneath the scleral flap) had
also been used. But it failed in the right eye
and the patient had developed a painful blind
eye.
www.bjophthalmol.com
His visual acuity was no light perception in
the right eye and 20/60 in the left. There was
right sided periorbital oedema, restricted
motility, and purulent discharge with matting
of the eyelashes. Rupture of the globe with
prolapse of the iris was evident along the
superior limbus. In the left eye the bleb was
well formed, and the IOP (Goldmann appla-
nation) was 12 mm Hg. The optic disc showed
a cup disc ratio of 0.4 with temporal pallor.
A diagnosis of panophthalmitis of the right
eye was made. As no response to intravenous
antibiotic treatment was observed over the
next 48 hours, evisceration was undertaken
with the patient’s informed consent. Staphylo-
coccus epidermidis was grown on culture.
Comment
Its efficacy notwithstanding, diode laser cy-
clophotocoagulation has been associated with
complications, although less frequent than
with other cyclodestructive procedures. Con-
junctival burns, corneal decompensation,
neurotrophic corneal defects, uveitis, IOP
spikes, phthisis (0.5%), chronic hypotony
(1%), macular pucker (0.5%), intraocular
haemorrhage (0.5%), visual loss, and malig-
nant glaucoma have all been reported.1 5–8
Possible correlation of outcome with perilim-
bal pigmentation, and the hearing of audible
“pop,”6 especially in patients with dark brown
irides,4 has been suggested.
Scleral thinning is a recognised risk factor
for complications, but scleral perforation
following this procedure has been reported
earlier in one patient only.9 This patient had
scleral thinning following previous cataract
surgery. Possibly, the sharp probe edge had cut
conjunctival vessels causing bleeding and
contamination of the probe tip. Thin adherent
debris was then carbonised allowing the laser
tip temperature to rise to 300°C, sufficient to
cause scleral perforation. This report led to
redesigning of the probe tip to protect the
vascular structures from its sharp edges.
Our patient was treated as part of a stand-
ard protocol using the modified G probe, and
bleeding did not occur. The perforation could
have resulted from mechanical pressure, or
the effect of the laser on the thinned out
sclera, which the patient probably had as a
result of buphthalmos and previous ocular
surgery.9 There are no known “corrective fac-
tors” to utilise in eyes with severe scleral thin-
ning, and if treatment is absolutely necessary,
a 40% lower energy should be used,10 and
areas of thinning and possible wound dehis-
cence, such as the bleb, should be avoided. We
emphasise caution in undertaking contact
diode laser trans-scleral cyclophotocoagula-
tion in operated eyes with thin sclera.
P Venkatesh, M Gogoi, R Sihota,
H Agarwal
Dr Rajendra Prasad Centre for Ophthalmic
Sciences, All India Institute of Medical Sciences,
New Delhi, India
Correspondence to: Dr Pradeep Venkatesh, Dr
Rajendra Prasad Centre for Ophthalmic Sciences,
All India institute of Medical Sciences, Ansari
Nagar, New Delhi 110029, India;
venkyprao@yahoo.com
Accepted for publication 5 Spetember 2002
References
1 Bloom PA, Tsai JC, Sharma K, et al.
“Cyclodiode” trans-scleral diode laser
cyclophotocoagulation in the treatment of
advanced refractory glaucoma.
Ophthalmology 1997;104:1508–20.
2 Kosoko O, Gaasterland DE, Pollack IP, et al.
Long-term outcome of initial ciliary ablation
PostScript
with contact diode laser transscleral
cyclophotocoagulation for severe glaucoma.
The Diode Laser Ciliary Ablation Study Group.
Ophthalmology 1996;103:1294–302.
3 Becker M, Funk J. Diode laser
cyclophotocoagulation as the primary surgical
intervention in glaucoma [article in German].
Ophthalmologe 2001;98:1145–8.
4 Martin KR, Broadway DC. Cyclodiode laser
therapy for painful, blind glaucomatous eyes.
Br J Ophthalmol 2001;85:474–6.
5 Yap-Veloso MI, Simmons RB, Echelman DA,
et al. Intraocular pressure control after contact
transscleral diode cyclophotocoagulation in
eyes with intractable glaucoma. J Glaucoma
1998;7:319–28.
6 Azuara-Blanco A, Dua HS. Malignant
glaucoma after diode laser
cyclophotocoagulation. Am J Ophthalmol
1999;127: 467–9.
7 Hennis HL, Stewart WC. Semiconductor
diode laser transscleral cyclophotocoagulation
in patients with glaucoma. Am J Ophthalmol
1992;113:81–5.
8 Johnson SM. Neurotrophic corneal defects
after diode laser cycloablation. Am J
Ophthalmol 1998;126:725–7.
9 Sabri K, Vernon SA. Scleral perforation
following trans-scleral cyclodiode. Br J
Ophthalmol 1999;83:502–3
10 Palmer DJ, Cohen J, Torczynski E, et al.
Transscleral diode laser
cyclophotocoagulation on autopsy eyes with
abnormally thinned sclera. Ophthalmic Surg
Lasers 1997;28:495–500.
Bilaminar interepithelial bodies
within fingerprint dystrophy-like
changes in bilateral iridocorneal
endothelial syndrome
In most cases the iridocorneal endothelial
(ICE) syndrome affects women unilaterally
and shows endothelial degeneration, thicken-
ing of Descemet’s membrane, iris atrophy, and
glaucoma. The spectrum of the ICE syndrome
includes the Chandler’s syndrome, the essen-
tial iris atrophy, and the Cogan-Reese syn-
drome. We present a unique case of an ICE
syndrome, which we would subclassify as
Chandler’s syndrome, with concomitant fin-
gerprint dystrophy and multiple interepithe-
lial bilaminar bodies within the fingerprint-
like striae.
Case report
Ten years earlier a now 45 year old patient
presented with a bilateral ICE syndrome and
fingerprint dystrophy. On slit lamp micros-
copy the epithelial layer showed fingerprint
lines, while the endothelial layer had a “ham-
mered” appearance. Iris atrophy, corectopia,
ectropium uveae and anterior synechiae were
obvious (Fig 1A) and progressive in both eyes,
nevertheless the intraocular pressure and the
visual acuity were normal. Eight years later
both corneas developed oedema (Fig 1B) and
the left eye displayed cataract with reduction
of visual acuity from 1.0 to 0.25 and 0.9 to 0.7
in the left and right eye, respectively. Com-
bined perforating keratoplasty and extracap-
sular cataract extraction were performed on
the left eye in June 1998. None of the patient’s
family members had a similar condition.
The histological examination of the paraffin
embedded corneal specimen revealed
fingerprint-like striae containing small ba-
sophilic deposits between the cells (Fig 2A).
The van Kossa stain for calcium was negative
and no hydroxyapatite crystals were seen. At
the ultrastructural level the deposits were
found to be round and bilaminar showing an
electron dense core in the centre (Fig 2B).
Some of the deposits coalesced to form oval
PostScript
Figure 1 (A) The left eye in 1990 with iris
atrophy, corectopia, ectropium uveae, and
anterior synechiae. (B) The left eye in 1998
with progression of the pathological
changes.
Figure 2 (A) Fingerprint-like stria
containing small basophilic deposits between
the cells (toluidine blue, ×400). (B) The
cytoplasm of the epithelial cells was normal
without deposits in relation to the cell
membranes (uranyl acetate, lead citrate, bar
= 250 nm).
structures. The cytoplasm of the adjacent epi-
thelial cells was normal without deposits in
relation to the cell membranes. Descemet’s
membrane was not thickened, the endothelial
cells possessed microvilli, their mitochondria
were degenerated, and intercellular interdigi-
tations were pronounced.
Comment
We consider our case as one of Chandler’s
syndrome, which is part of the spectrum of
the ICE syndrome, owing to obvious morpho-
logical changes of the iris despite non-specific
endothelial changes. The striking features of
Chandler’s syndrome are corneal endothelial
dystrophy and subsequent oedema.1 Corecto-
pia, glaucoma, and iris atrophy are less
compared to the two other ICE syndromes—
that is, the essential iris atrophy and the iris-
naevus syndrome, possibly owing to differ-
ences in endothelial proliferative activity.2
Our case is unique in several aspects. Firstly,
our patient is a man and is affected bilaterally.
Most often women are affected unilaterally by
ICE syndrome, nevertheless an exception of
essential iris atrophy with bilateral changes
have been described.3
Secondly, an epithelial dystrophy—namely,
a fingerprint dystrophy, is present in addition
to the ICE associated changes. To our knowl-
edge this association has not been described
in the literature before.
Thirdly, the fingerprint striae contain bil-
aminar deposits with an electron dense core.
Several types of deposits have been described
in association with epithelial dystrophies.
Patches of “unknown substance” were no-
ticed in fingerprint dystrophy along basal
plasmalemmas of the basal epithelial cells
with no similarity to the bilaminar structures
described here.4 Broderick et al mentioned
intercellular dense oblong bodies which were
embedded in condensed matrix and were
much smaller than the deposits in our case.5
In a cornea with a Meesmann dystrophy
intraepithelial “peculiar electron dense sub-
stance” were shown, also with a totally differ-
ent appearance compared to that seen in the
fingerprint striae of the specimen we present
here. In a patient with ocular tumoral
calcinosis, nodules from the palpebral con-
junctiva contained calcified “spherules,”6
which show some similarity to our—
nevertheless, uncalcified—deposits. Our de-
posits were located within the fingerprint
striae, close to the epithelium. It seems to be
obvious that the epithelial cells must have
produced those bilaminar bodies, since no
other cell type was present nearby, but there
was no evidence of extrusion from the basal
cells.
Our case shows that ICE syndrome can
occur bilaterally in men, and can be associated
with a fingerprint-like dystrophy. Further
studies should be carried out to confirm our
electron microscopical observation of bilami-
nar deposits within fingerprint-like striae and
directed towards an analysis of their chemical
composition of the extracellular material and
origin.
Acknowledgements
We thank Mrs Annegret Mattes and Mrs Dorothy A
Aitken for providing electron microscopic assistance.
This case was presented as a poster at the meeting
of the Association for Research and Vision in
Ophthalmology in Fort Lauderdale in May 2001.
C Auw-Haedrich
Universitäts-Augenklinik Freiburg, Germany
U Sengler
Universitäts-Augenklinik Düsseldorf, Germany
W R Lee
Department of Pathology, University of Glasgow,
Scotland, UK
Correspondence to: Dr Claudia Auw-Haedrich,
Universitäts-Augenklinik, Killianstrasse 5, D-79106
Freiburg, Germany; auw@aug.ukl.uni-freiburg.de
Accepted for publication 9 September 2002
References
1 Chandler PA. Atrophy of the stroma of the
iris: esndothelial dystrophy, corneal edema,
509
and glaucoma. Am J Ophthalmol
1956;41:607–15.
2 Campbell DG, Shields MB, Smith TR. The
corneal endothelium and the spectrum of
essential iris atrophy. Am J Ophthalmol
1978;86:317–24.
3 Kaiser-Kupfer M, Kuwabara T, Kupfer C.
Progressive bilateral essential iris atrophy. Am
J Ophthalmol 1977;83:340–6.
4 Rodriguez MM, Fine BS, Laibson PR, et al.
Disorders of the corneal epithelium. A
clinicopathologic study of dot, geographic,
and fingerprint patterns. Arch Ophthalmol
1974;92:475–82.
5 Brodrick JD, Dark AJ, Geoffrey WP.
Fingerprint dystrophy of the cornea. Arch
Ophthalmol 1974;92:483–9.
6 Ghanchi F, Ramsay A, Coupland S, et al.
Ocular tumoral calcinosis. Arch Ophthalmol
1996;114:341–5.
Fine retinal crystalline deposits
observed by confocal scanning
laser ophthalmoscopic
examination using infrared light
Bietti’s crystalline retinopathy is a tapetoreti-
nal degeneration characterised by numerous
tiny sparkling yellow-white spots mainly
located in the deeper layer of the posterior
pole retina.1 Since crystalline deposits tend to
become small and decrease in number over
time,2 3 the fundus appearance becomes
indistinguishable from other retinal dystro-
phies with time.4 The advent of confocal
scanning laser ophthalmoscope (SLO) with
infrared light has enabled high sensitive
examination of the deep layer of the retina.5
We studied the deep retinal abnormalities in
a case with a typical Bietti’s crystalline retin-
opathy and in another case with myopic cho-
rioretinal atrophy throughout the posterior
pole. Consequently, we detected numerous
fine crystalline deposits in both cases, which
were not detected with other funduscopic
examinations.
Case reports
Case 1
A 52 year old man was referred to our hospi-
tal by his ophthalmologist who found an
abnormal fundus appearance. There was no
consanguinity. Best corrected visual acuity
was 20/24 right eye and 20/50 left eye. No
abnormalities were found in the anterior
segment and media. Fine crystalline deposits
were not detected in the corneal stroma of
either eye. Funduscopic examination re-
vealed numerous fine reflective crystalline
deposits throughout the posterior pole and
mid-peripheral retina of both eyes. Most of
these deposits were in the deep retina and
subretina but some deposits were superficial.
Goldmann perimetry showed relative scoto-
mas in a zone within 30–40 degrees of the
central field in both eyes. Fluorescein angio-
graphy of both eyes showed island-like
hypofluorescence lesions surrounded by hy-
perfluorescent lesions in the early phase.
Indocyanine green angiography also showed
the atrophy of the choriocapillaris in the pos-
terior pole. Crystalline deposits did not show
any fluorescence in either fluorescein or
indocyanine green angiography.
The retinal crystalline deposits were more
clearly visible with the fundus examination by
SLO (Rodenstock, Germany) using infrared
light compared to the routine funduscopic
examinations (Fig 1). Each crystal deposit
appeared larger in size. In addition, numerous
fine crystal deposits were evident, which were
not observed by conventional funduscopic
examinations including ophthalmoscopy and
www.bjophthalmol.com
510
Figure 1 Ocular fundus of case 1. Fundus
photograph (A), grey scale image of the
same fundus photograph (B) and SLO image
with infrared light (C) are shown. Note that
the crystalline deposits are most evident with
the infrared imaging.
slit lamp biomicroscopy using a contact lens.
When the source of light was moved, the
crystalline deposits scattered light and be-
came more prominent.
Case 2
A 52 year old woman visited our hospital
complaining of gradual visual loss in both
eyes. She also noticed night blindness since
childhood. Her parents were both high myopic
and consanguineous. The extracapsular cata-
ract extraction surgery was performed with-
out complications in both eyes at the age of
43, and referring ophthalmologist reported
that her fundus showed extensive chorioreti-
nal atrophy in both eyes and that best
corrected visual acuity was 20/1000 right eye
and 20/1000 left eye after the cataract surgery.
At initial visit to our hospital, best corrected
visual acuity was right eye 20/200 and left eye
20/1000. Conjunctiva, cornea, anterior cham-
ber, and vitreous were normal in both eyes
Figure 2 Ocular fundus of case 2. Fundus photograph (A), grey scale image of the same
fundus photograph (B) and SLO image with infrared light (C). Magnified image of the boxed
area is shown in (D). Note that numerous crystalline deposits are observed with the SLO
imaging.
www.bjophthalmol.com
and no fine crystal deposits were found at
limbs of either cornea. Both eyes were aphakic
and the posterior lens capsules were intact but
relatively opaque. Funduscopic examination
revealed the posterior staphyloma and the
extensive atrophy of the retinal pigment
epithelium and choriocapillaris extending
from the posterior to mid-peripheral retina in
both eyes. Optic disc showed myopic appear-
ance. Retinal vessels and peripheral retina
showed normal appearance. Standard elec-
troretinogram was non-recordable. Gold-
mann perimetry showed marked constriction
of all isoptres and loss of visibility of I-4-e
isoptre for both eyes. During a 5 year follow
up, visual acuity and the fundus appearance
were stationary. Fluorescein angiography per-
formed at the age of 57 demonstrated
hypofluorescence throughout the posterior
pole in the early phase and diffuse hyperfluo-
rescence in the later phase. Indocyanine green
angiography disclosed the loss of background
veil-like fluorescence throughout the poste-
rior pole due to the choriocapillaris atrophy.
Fundus examination by the confocal SLO
with infrared light disclosed numerous tiny
crystalline deposits in the posterior pole (Fig
2), which were not evident with other fundu-
scopic examinations, including ophthalmos-
copy and biomicroscopy with a contact lens,
or with fluorescein or indocyanine green
angiographic studies. The deposits scattered
light when the source of light was moved to
cause them sparkle, similar to the fine
crystalline deposits observed in the fundi of
case 1.
Amino acid analysis and other examina-
tions did not show any abnormal value.
Comment
The diagnosis of Bietti’s crystalline retin-
opathy in case 1 is based on the retinal
crystalline and supported by the characteris-
tic island-like hypofluorescence observed by
fluorescein angiography.2 Fundus examina-
tion by SLO with the infrared light disclosed
numerous tiny crystalline deposits, which
were not observed by conventional fundus
examinations. Thus, in Bietti’s crystalline
retinopathy, there are more numerous accu-
mulation of retinal crystalline deposits than
can be observed by funduscopic examina-
tions.
In case 2, numerous tiny retinal crystalline
deposits were detected by SLO with infrared
light, although no other examinations dis-
closed these crystalline deposits. We have not
observed such retinal crystalline deposits in
other cases with myopic chorioretinal atrophy,
suggesting the accumulation of crystalline
deposits is not a generalised feature of myopic
chorioretinal degeneration. Crystalline depos-
its, prerequisite for the diagnosis of Bietti’s
crystalline retinopathy, are difficult to detect
in advanced cases.2–4 Since crystalline deposits
do not block or transmit fluorescence in
PostScript
fluorescein2 or indocyanine green angio-
graphy, diagnosis of Bietti’s crystalline retin-
opathy in advanced cases without fundu-
scopically apparent crystalline deposits have
been established only when family history
was contributory.4 Because the presence of
crystalline material in the peripheral
lymphocyte4 was not observed by transmis-
sion electron microscopy, whether case 2 can
be categorised as Bietti’s crystalline retin-
opathy or a distinct clinical entity awaits fur-
ther investigation. Additionally, to our knowl-
edge, Bietti’s crystalline retinopathy with
degenerative myopia has not been reported in
the literature. However, the presence of
parental consanguinity of this case might
suggest an autosomal recessive inheritance,
similar with some reported cases with Bietti’s
crystalline retinopathy, and the striking re-
semblance of the morphology of the tiny crys-
talline deposits observed by SLO using infra-
red light between the two cases may support
that underlying pathogenesis is similar.
Y Yanagi, Y Tamaki, H Fukushima
Department of Ophthalmology, University of Tokyo
School of Medicine, 7-3-1 Hongo, Bunkyo-ku,
Tokyo 113-8655, Japan
Correspondence to: Yasuo Yanagi, Department of
Ophthalmology, University of Tokyo School of
Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo
113-8655, Japan; yanagi-tky@umin.ac.jp
Accepted for publication 25 September 2002
References
1 Bietti GB. Su alcune forme atipiche o rare di
degenerazione retinica (degenerazione
tappetoretiniche e quadri morbosi similari).
Boll Oculist 1937;16:1159–244.
2 Yuzawa M, Mae Y, Matsui M. Bietti’s
crystalline retinopathy. Ophthalmic Paediatr
Genet 1986;7:9–20.
3 Bernauer W, Daicker B. Bietti’s
corneal-retinal dystrophy. A 16-year
progression. Retina 1992;12:18–20.
4 Kaiser-Kupfer MI, Chan CC, Markello TC, et
al. Clinical biochemical and pathologic
correlations in Bietti’s crystalline dystrophy.
Am J Ophthalmol 1994;118:569–82.
5 Manivannan A, Kirkpatrick JN, Sharp PF, et
al. Clinical investigation of an infrared digital
scanning laser ophthalmoscope. Br J
Ophthalmol 1994;78:84–90.
Tonic pupils from giant cell
arteritis
The tonic pupil is characterised by poor
reactivity to light, a slow tonic constriction
and redilation to a near target, and supersen-
sitivity to topical dilute pilocarpine.1 Most
instances do not have an identifiable cause;
however, a variety of conditions including
herpes zoster, orbital trauma including sur-
gery, and paraneoplastic syndromes may
result in tonic pupils. The mechanism(s)
which produce tonic pupils are not known,
although axonal loss within the ciliary gan-
glion is generally believed to result in super-
sensitivity of the iris sphincter muscle. Giant
cell arteritis (GCA) has been reported as an
uncommon cause of tonic pupils.2 Further-
more, the cause of tonic pupils in GCA is
unknown and direct evidence for an ischae-
mic cause is not convincing.
We used orbital colour Doppler imaging to
study orbital and ocular blood flow in a
patient with GCA, unilateral visual loss, and
bilateral tonic pupils.
Case report
A 58 year old woman presented with visual
loss of the left eye for 2 days. She had had
PostScript
Figure 1 (A) The right optic disc and
macula are normal. (B) The left optic disc is
pale and swollen. Scattered cotton wool
infarcts are seen within the macula.
neck discomfort and intermittent jaw claudi-
cation over the previous 2 weeks. She had no
past medical or ocular problems.
Visual acuity was 20/20 in the right eye and
no light perception in the left eye. The right
pupil reacted briskly to light, with a normal
consensual response, the left was amaurotic.
She identified all of the Ishihara pseudoiso-
chromatic colour plates with the right eye,
and automated perimetry of the right eye was
normal. Extraocular motility was normal.
Funduscopy of the right eye (Fig 1A) was
normal, and the left optic disc was pale and
swollen, with retinal cotton wool infarcts
within the retina, chiefly within the left
macula (Fig 1B). The superficial temporal
arteries were supple, pulsatile, and non-
tender. The Westergren erythrocyte sedimen-
tation rate (ESR) was 114 mm in the first
hour.
She was hospitalised and received intra-
venous methylprednisolone 250 mg every 6
hours. Temporal artery biopsy was positive for
GCA, including the presence of numerous
giant cells. After 12 doses of methylpred-
nisolone she was discharged on 80 mg of oral
prednisone per day.
Six days later her visual acuity remained
20/20, and with a +2.00 dioptre add over a
plano lens she read J1 print at 13 inches with
the right eye. Automated perimetry and
funduscopy of the right eye remained normal.
However, the right pupil now reacted slug-
gishly to light (Fig 2A) while the left
remained amaurotic. In addition, there was
light near dissociation in both eyes, with
bilateral tonic constriction to a near target
followed by slow redilation. Slit lamp exam-
ination on the right revealed an irregular
pupil (Fig 2B) with sectoral iridoplegia. Both
pupils constricted following the instillation of
topical 0.125% pilocarpine (Fig 2C).
She remained asymptomatic in her right
eye and neuro-ophthalmic examination was
unchanged 12 days later, except that her right
pupil no longer reacted to direct light. Both
pupils constricted slowly and tonically to a
near target. Orbital colour Doppler imaging
revealed no blood flow in the left central reti-
nal artery (CRA), and blood flow in the right
CRA was attenuated at 4.7 cm/s (normal 10
cm/s). There was no flow detected in the short
posterior ciliary arteries of the left eye, and
attenuated flow in the short posterior ciliary
arteries on the right. Although her ESR fell to
4 mm in the first hour and her jaw claudica-
tion resolved, the prednisone dose was main-
tained because of the attenuated blood flow
within the right orbit. Her vision remained
unchanged and her pupils remained non-
reactive to light but with denervation super-
sensitivity, confirmed by constriction to
0.125% pilocarpine, 2 months after the onset
of visual loss.
Comment
Five earlier reports have documented tonic
pupils in patients with GCA. Davis and
coworkers described a 69 year old woman who
developed polymyalgia and anisocoria.2 The
involved pupil constricted to 2.5% metha-
choline. In another report of tonic pupils from
GCA mild supersensitivity to 0.1% pilocarpine
was noted; however, the patient had counting
fingers vision in each eye from bilateral optic
neuropathy, suggesting that the light near
dissociation may have been caused by bilat-
eral afferent dysfunction.3 The authors cited
pathological studies which have suggested
ischaemia of the extraocular muscles as a
cause of ophthalmoplegia in some patients
with GCA,4 and they implied that ischaemia
was the cause of tonic pupils in their patient,
although the site of damage was not specified.
Currie and Lessell reported a 63 year old
woman who had jaw claudication and loss of
Figure 2 (A) The right pupil reacts
sluggishly to light. (B) The right pupil is oval
and the contour of the pupillary margin is
asymmetric. (C) Both pupils constricted 30
minutes after instilling topical 0.125%
pilocarpine.
511
vision bilaterally from sequential anterior
ischaemic optic neuropathy owing to biopsy
proved GCA.5 After losing vision in each eye
she developed bilateral tonic pupils which
constricted markedly to 0.08% pilocarpine.
These authors suggested that impaired orbital
blood flow, including the blood supply to the
ciliary ganglia or its postganglionic ciliary
nerves, resulted in loss of axons and denerva-
tion supersensitivity, although no orbital
blood flow studies were performed. They
speculated that tonic pupils in patients with
GCA may be overlooked because of the sever-
ity of visual loss.
Other authors have suggested that ischae-
mic damage may not be the sole cause of tonic
pupils in patients with GCA. Bilateral tonic
pupils with conjunctival injection were the
only ocular manifestations in a 60 year old
woman with biopsy proved GCA.6 Because
there was no other clinical evidence of orbital
or ocular ischaemia the authors postulated
that damage to the ciliary ganglion may be
mediated by an immunological mechanism.
However, they did not quantitate the orbital or
ocular blood flow, and our patient shows that
clinical signs of ischaemia, except for tonic
pupils, may be absent despite markedly
decreased orbital blood flow.
Wilhelm reported five patients with bilat-
eral visual loss from anterior ischaemic optic
neuropathy or central retinal artery occlusion
and tonic pupils from GCA.7 Assessment of
orbital blood flow was performed using ultra-
sonography in three of these patients. In two
patients no flow was noted in the supratroch-
lear arteries, while blood flow in the third was
normal. The author noted that the ultrasono-
graphic results supported ischaemia as a
cause for the tonic pupils. In the patient with
normal orbital blood flow, ischaemia below
the resolution of ultrasonography was pre-
sumed to cause the tonic pupils.
The blood supply to the ciliary ganglion
arises from several sources. Eliskova studied
18 human cadaveric orbits after injection of
dye into the internal carotid artery.8 The
ciliary ganglion was supplied with blood from
one to four arteries, with the posterior lateral
ciliary artery and the lateral muscular artery
the most common sources, followed by the
ophthalmic and central retinal artery. The
vasculature of the ganglion itself is composed
of a network of capillaries.
Orbital colour Doppler imaging is a non-
invasive way to quantitate blood flow to the
orbit and eye. Decreased blood flow in the
ophthalmic artery, central retinal artery, and
short posterior ciliary arteries may be de-
tected on orbital colour Doppler imaging in
patients with GCA.9 Markedly reduced blood
flow was found with orbital colour Doppler
imaging in both orbits in our patient, despite
her visual loss being unilateral. We were
unable to find a previous report of bilateral
tonic pupils in a patient with unilateral visual
loss from GCA. Although anterior segment
ischaemia can cause loss of iris sphincter tone,
we do not believe that the pupillary findings
in our patient resulted from iris ischaemia.
Iris ischaemia would result in poorly reactive
pupils to both light and accommodation,
without a tonic near reaction or denervation
hypersensitivity. Therefore, the findings in our
patient strongly suggest that the tonic pupils
resulted from ischaemic damage to the ciliary
ganglion or the postganglionic ciliary nerves
which are responsible for pupillary constric-
tion.
The authors do not have any proprietary interests in
any of the contents of this manuscript.
Dr Foroozan is supported by the Heed Ophthalmic
Foundation, Cleveland, OH, USA.
www.bjophthalmol.com
512
R Foroozan, L M Buono, P J Savino,
R C Sergott
Neuro-Ophthalmology Service, Wills Eye Hospital,
Thomas Jefferson University, 900 Walnut Street,
Philadelphia, PA 19107, USA
Correspondence to: P J Savino; pjsavino@aol.com
Accepted for publication 12 September 2002
References
1 Bourgon P, Pilley FJ, Thompson HS.
Cholinergic supersensitivity of the iris sphincter
in Adie’s tonic pupil. Am J Ophthalmol
1978;85:373–7.
2 Davis RH, Daroff RB, Hoyt WF. Tonic pupil
after temporal arteritis. Lancet 1968;1:822.
3 Bronster DJ, Rudolph SH, Shanzer S.
Pupillary light-near dissociation in cranial
arteritis. Neuro-Ophthalmology
1982;3:65–70.
4 Barricks ME, Traviesa DB, Glaser JS, et al.
Ophthalmoplegia in cranial arteritis. Brain
1977;100:209–21.
5 Currie J, Lessell S. Tonic pupil with giant cell
arteritis. Br J Ophthalmol 1984;68:135–8.
6 Coppeto JR, Greco T. Mydriasis in giant-cell
arteritis. J Clin Neuro-ophthalmol
1989;9:267–9.
7 Wilhelm H. Pupillotonie durch ischamie
[Tonic pupil caused by ischemia]. Fortschr
Ophthalmol 1989;86:380–2.
8 Eliskova M. Blood vessels of the ciliary
ganglion in man. Br J Ophthalmol
1973;57:766–72.
9 Aburn NS, Sergott RC. Orbital colour
Doppler imaging. Eye 1993;7:639–47.
Frozen cucumber as a mount for
processing vitreoretinal
specimens
Vitreoretinal specimens are extremely diffi-
cult to process as a frozen specimen because of
their small size and tendency to roll up.1 How-
ever, in order to perform immunohisto-
chemical tests it is sometimes necessary to
have frozen specimens as antigens may be
destroyed if a fixative agent is used.
Dua et al2 and Scott et al3 suggested the use
of frozen cucumber as a mount for conjuncti-
val and corneal tissue; we modified this
method for vitreoretinal specimens. We de-
scribe our technique and provide examples of
our results.
Case report
Fresh cucumber (obtained from a greengro-
cer) was cut into small (1 cm3) blocks; the part
devoid of seeds was used. We found that with
cucumbers older than 2 days the membranes
did not adhere sufficiently well. These blocks
were then stored at 4°C until required. Pig
eyes were obtained and stored at 4°C until
required. Subsequently, the eye was placed
under the dissecting microscope and basic salt
solution injected in through the vitreous cav-
ity to enable easier dissection. The cornea was
Figure 1 The biopsy cucumber unit.
www.bjophthalmol.com
Figure 2 Diabetic membrane on cucumber
mount stained with haematoxylin and eosin.
removed and a vitrectomy performed. The
retinal specimens were stained with Indian
ink in order to facilitate subsequent localisa-
tion.
Following vitrectomy, membranes were re-
moved from humans (these included diabetic
membranes, subretinal neovascular mem-
branes, and epiretinal membranes); they were
initially placed in Hartmann’s solution. Sub-
sequently, they were placed on the cucumber
under a dissecting microscope; it was possible
to place the membrane flat without it rolling
up because of the texture of the cucumber.
These membranes were also stained with
Indian ink before placing on the cucumber.
The cucumber with the membrane on its
“side” surface was placed in an aluminium
foil cup and covered with a cryomatrix of
Tissue-Tek OCT compound (Fig 1). The foil
cup was then put in a plastic container and
the contents flash frozen in liquid nitrogen.
The membranes were cut with a cryostat in
4–5 µm sections. We stained one slide from
each specimen with haematoxylin and eosin
and performed immunohistochemistry on the
others (Figs 2 and 3).
No specimens were lost while performing
this technique of processing specimens.
We were able to maintain the orientation of
the specimens and managed to obtain suffi-
ciently satisfactory specimens to perform our
immunohistochemical studies. The use of
Indian ink allowed us to locate the specimen
easily when cutting sections. Unlike previous
studies we found that the specimen attached
to the cucumber without the use of albumin.
Comment
Swan and Davis first described the biopsy
cucumber unit for processing cervical
specimens.4 Frozen cucumber has been de-
scribed as a mount for bladder and lung
biopsies.5 6
Ocular tissues that have been processed in
this fashion are conjunctiva and cornea.2 7
Whittle et al described a technique using
Figure 3 Diabetic membrane on cucumber
mount stained with CD31.
PostScript
cucumber as a mount for processing cadaveric
human retina,8 which enabled indirect
immunofluorescence studies.
Retinal specimens are difficult to process
because of their size, tendency to roll up and,
hence, difficult orientation. Nevertheless, it is
necessary to process specimens in this way to
perform certain immunohistochemistry tech-
niques.
We suggest that cucumber is a suitable
mount for vitreoretinal membranes that are
required as frozen specimens for immunohis-
tochemistry. It should be noted that most
modern immunohistochemistry may be per-
formed on fixed tissue.
Presented at British Association of Ophthalmic
Pathologists Annual Meeting, Dunchurch, March
2002
Each author states that he has no proprietary
interest in the development or marketing of any
product used in this study.
S Banerjee, C Shaikh, G R Wallace,
P I Murray
Academic Unit of Ophthalmology, University of
Birmingham, Birmingham, UK
R A H Scott
Vitreoretinal Unit, City Hospital NHS Trust,
Birmingham, UK
Correspondence to: Professor P I Murray, Academic
Unit of Ophthalmology, Division of Immunity and
Infection, Birmingham and Midland Eye Centre,
Sandwell and West Birmingham Hospitals NHS
Trust, City Hospital, Dudley Road, Birmingam
B18 7QU, UK; P.I.Murray@bham.ac.uk
Accepted for publication 13 September 2002
References
1 Hiscott P, Wong D, Grierson I. Challenges in
ophthalmic pathology: the vitreoretinal
membrane biopsy. Eye 2000;14,549–59.
2 Dua HS, Gomes JAP, Singh A, et al.
Fresh-frozen cucumber as a mount for
conjunctival and corneal tissue in
cryomcirotomy. Arch Ophthalmol
1994;112:1139–40.
3 Scott RA, Lauweryns B, Snead DM, et al.
E-cadherin distribution and epithelial
basement membrane characteristics of the
normal human conjunctiva and cornea. Eye
1997;11 ( Pt 5):607–12.
4 Swan RW, Davis HJ. The biopsy-cucumber
unit. A method to improve tissue orientation.
Obstet Gynecol 1970;36:803–5.
5 Watson RA, Fitzwater JE, Deshon GE Jr, et al.
Biopsy-cucumber unit: improved method for
preparing bladder biopsy specimens. Urology
1984;23:392–3.
6 Brodersen BW, Kelling CL. A cucumber
mount for processing lung biopsy specimens
from calves. J Vet Diagn Invest
1996;8:518–9.
7 Erie JC, Collyer SK, Campbell RJ. Dehydrated
cucumber slice as a mount for conjunctival
biopsy specimens. Am J Ophthalmol
1985;99:539–41.
8 Whittle RM, Wallace GR, Whiston RA, et al.
Human antiretinal antibodies in toxoplasma
retinochoroididtis. Br J Ophthalmol
1998;82:1017–21.
Inflammatory optic neuropathy
as the presenting feature of
herpes simplex acute retinal
necrosis
Acute retinal necrosis (ARN) is a rare but
serious ophthalmic manifestation of infection
by the herpesvirus family. In the immuno-
competent, the viral agent most frequently
identified is varicella zoster (VZV) followed by
herpes simplex (HSV-1 and HSV-2) and rarely
PostScript
Figure 1 (A) Colour photograph of right posterior pole (case 1, 20 year old man) showing
marked swelling of the right optic disc and several retinal haemorrhages; (B) fundus
fluorescein angiography of same eye mid-venous phase showing peripheral retinal occlusive
arteritis and ischaemia; (C) and (D), persistent disc swelling and patches of acute retinal
necrosis.
cytomegalovirus and Epstein-Barr virus. The
condition, which may present with synchro-
nous or metachronous systemic or cerebral
herpetic infection (encephalitis) is now recog-
nised to occur at all ages,1 though VZV tends
to affect the more elderly and herpes simplex
the young.
The majority of patients present with a
short history of blurred vision, floaters, and
mild ocular discomfort. In the immuno-
competent, clinical examination reveals one
or more well demarcated foci of retinal necro-
sis outside the major arterial arcades typically
with a dense vitritis that may preclude a
detailed view of the posterior segment.
Disease progression is rapid with coalescence
and posterior extension of areas of retinitis.
Often there is an occlusive, sometimes haem-
orrhagic, vasculitis either adjacent to areas of
retinitis or at the optic nerve. The visual prog-
nosis for untreated cases is very poor.2
We report three immunocompetent pa-
tients with no evidence of concurrent herpes-
virus infection in whom ipsilateral optic neu-
ritis associated with a panuveitis was the
presenting feature of their acute retinal
necrosis syndrome.
Case reports
Case 1
A 20 year old healthy white male, with no his-
tory of herpesvirus infection, presented with a
10 day history of progressive blurred right
vision with floaters, significant periocular dis-
comfort, and pain on ocular motility. Acuity
was 20/200 right eye, 20/20 left eye, with a
right relative afferent pupillary defect
(RAPD). There was swelling of the right optic
nerve (Fig 1A) together with increasing non-
granulomatous pan uveitis. The left eye was
unaffected. Fluorescein angiography (Fig 1B)
showed marked optic disc hyperfluorescence
and peripheral retinal ischaemia. Seven days
later (Fig 1C and D), several enlarging foci of
retinitis, typical of ARN, developed in the reti-
nal mid-periphery. PCR of a vitreous biopsy
amplified HSV-1. There was no further reduc-
tion in visual acuity following intravitreal
foscarnet and encephalitic doses (10 mg/kg
three times daily) of intravenous aciclovir for
10 days.
Case 2
A 47 year old healthy white female, with no
history of prior herpesvirus infection, pre-
sented with a 3 day history of progressive
blurred left vision. She also complained of
significant periocular discomfort exacerbated
by eye movement. Acuity was 20/40 left eye,
20/15 right eye, with a left RAPD. There was
swelling of the left optic nerve head together
with a moderate non-granulomatous panu-
veitis. The right eye was unaffected. Fluores-
cein angiography showed marked optic disc
hyperfluorescence and peripheral retinal is-
chaemia. Six days later, several enlarging foci
of retinitis, typical of ARN, developed in the
inferior retinal periphery. Polymerase chain
reaction (PCR) of a vitreous biopsy amplified
HSV-1. There was no further reduction in
visual acuity following encephalitic doses (10
mg/kg three times daily) of intravenous
aciclovir for 10 days.
Case 3
An 81 year old white male, who had suffered
herpes simplex meningoencephalitis and syn-
chronous right acute retinal necrosis (acuity
reduced to 20/200) 17 years earlier, presented
with a 2 day history of profound reduction in
left acuity to perception of light only. Clinical
findings included a left RAPD, haemorrhagic
optic disc swelling, and increasing hyperten-
sive panuveitis. Five days later, three demar-
cated areas of retinal necrosis appeared supe-
riorly in the same eye. PCR of a vitreous biopsy
513
amplified HSV-1. There was no further reduc-
tion in visual acuity following intravitreal
foscarnet and 10 days of encephalitic doses
(10 mg/kg three times daily) of intravenous
aciclovir.
Comment
There have been two previous reports in
immunocompromised patients of optic neuri-
tis preceding the development of ARN.3 4 In
these, preceding or concurrent cutaneous
herpes zoster infection suggested altered viral
behaviour in the context of deficient immu-
nity. Even in the healthy individual, there is
evidence of an immunogenetic predisposition
to the development of the disease.5 The novel
mode of presentation of our three immuno-
competent patients suggests they might share
a similar background of immunity that modi-
fies viral behaviour, thereby predisposing to
involvement of the optic nerve before the
development of retinitis. In this regard, it is
interesting that none of our cases developed
clinically evident encephalitis. This is a com-
mon accompaniment to human HSV-1 ARN
and observed in the Szily animal model in
which inoculation of the anterior chamber or
vitreous with HSV virus produces in ipsilat-
eral anterior uveitis, relative sparing of the
ipsilateral retina, and necrotising contra-
lateral chorioretinitis with encephalitis. It has
been shown that viral spread to the brain and
the fellow eye results from viral invasion of
the optic nerve.6 The ability of the host to
resist this appears determined by the animal’s
immune background.7
The recent report in a single case of ARN
subsequent to central retinal vascular ob-
struction in the fellow eye is most likely a
manifestation of a different VZV mediated
disease process8 but emphasises the ability of
members of the herpesvirus family to directly
invade blood vessel walls.
The combination of optic neuritis, periph-
eral retinal ischaemia, and panuveitis is very
unusual. In white patients, the differential
diagnosis would include conditions such as
sarcoidosis and demyelinating disease for
which steroid therapy is often routine and
may prove beneficial. This report highlights
that herpesvirus infections may also present
in this fashion. Since progression to profound
and irreversible visual loss is rapid, close daily
retinal examination, and early diagnostic
vitreous biopsy must be recommended for
these patients before commencement of im-
munosuppressives.
P J Francis, H Jackson, M R Stanford,
E M Graham
St Thomas’s Hospital, London, UK
Correspondence to: Peter Francis, St Thomas’s
Hospital, London, UK;
peterjamesfrancis@btopenworld.com
Accepted for publication 3 October 2002
References
1 Tan J, Byles D, Stanford M, et al. Acute
retinal necrosis in children caused by herpes
simplex virus. Retina 2001;21:344–7.
2 Walters G, James T. Viral causes of the acute
retinal necrosis syndrome. Curr Opin
Ophthalmol 2001;12:191–5.
3 Friedlander S, Rahhal F, Ericson L, et al.
Optic neuropathy preceding acute retinal
necrosis in acquired immunodeficiency
syndrome. Arch Ophthalmol
1996;114:1481–5.
4 Meenken C, Horn Gvd, Smet Md, et al.
Optic neuritis heralding varicella zoster virus
retinitis in a patient with acquired
immunodeficiency syndrome. Ann Neurol
1998;43:534–6.
www.bjophthalmol.com
514
5 Holland G, Cornell P, Park M, et al. An
association between acute retinal necrosis
syndrome and HLA-DQw7 and phenotype
Bw62, DR4. Am J Ophthalmol
1989;108:370–4.
6 Olson R, Holland G, Goss S, et al. Routes of
viral spread in the von Szily model of herpes
simplex virus retinopathy. Curr Eye Res
1987;6:59–62.
7 Zaltas M, Opremcak E, Hemady R, et al.
Immunohistopathologic findings in herpes
simplex virus chorioretinitis in the von Szily
model. Invest Ophthalmol Vis Sci
1992;33:68–77.
8 Kang S, Kim S. Optic neuropathy and central
retinal vascular obstruction as initial
manifestations of acute retinal necrosis. Jpn J
Ophthalmol 2001;45:425–8.
Cutaneous angiosarcoma of the
eyelids
Angiosarcoma of the eyelid is a rare poten-
tially life threatening tumour arising from the
vascular endothelium. Through the presenta-
tion of a case, the clinicopathological features
and management of this condition are de-
scribed.
Case report
A 69 year old white man presented with
multifocal red-blue maculopapular lesions
located in his right cheek and eyelids (Fig 1A).
The patient had first noticed the lesions 9
months previously. An incisional biopsy from
the upper eyelid was performed. In subse-
quent staging investigations no metastases
were found. The patient was otherwise in
good health and was HIV negative. He under-
went surgical resection of all clinically detect-
able lesions followed by adjuvant radio-
therapy (total dose 45 Gy). After 34 months of
follow up there was no evidence of local
recurrence or distant metastases.
Histopathologically, the specimens con-
sisted of skin and subcutaneous tissue. The
lesions proved to be well differentiated angi-
osarcoma characterised by interlacing blood
vessels lined by plump endothelial cells with
hyperchromatic nuclei. Using Ki-67, the pro-
liferation rate was assessed as 5%–7% (Fig
1B). Immunohistochemically, the tumour
cells showed a positive reaction for vimentin,
CD34 (human haemopoietic progenitor cell
antigen) and FVIII-RA. They were consist-
ently negative for S100 and HMB45 (results
not shown).
Comment
Cutaneous angiosarcoma is a distinct sub-
group of angiosarcomas most commonly seen
in the skin and superficial soft tissues in
patients over 55 years.1 The majority of these
tumours arise in the head and neck area, par-
ticularly the scalp. They are aggressive and
tend to recur locally and disseminate widely
with a 5 year survival of approximately 12%–
29%.1 2 To our knowledge, there are only seven
well documented cases involving the eyelid.3–8
Clinically, the lesions in our patient resembled
those previously reported in view of the
purple coloured maculopapular lesions, sug-
gesting a vascular origin.1 3–8 In 50% of cases
the lesions are multifocal.1 Often the lesions
are poorly defined and the true extent of dis-
ease may be difficult to determine
clinically.1 2 5 7 8 The differential diagnosis in-
cludes ecchymoses, capillary haemangioma,
melanoma, and Kaposi’s sarcoma. Advanced
lesions may ulcerate and atypical cases simu-
lating, cellulitis, rosacea and xanthelasma
have also been reported.3 5–8 Owing to lack of
clinical awareness and the varied presenta-
tion, diagnosis is often delayed.1
www.bjophthalmol.com
Figure 1 (A) Magnified view of lid lesions.
(B) Light micrograph showing well
differentiated angiosarcoma characterised by
distinct blood vessel formation. In areas,
malignant endothelial cells pile up producing
typical papillary projections into the lumina
(short arrow) (PAS ×200).
Histologically, angiosarcomas display a
spectrum from well to poorly differentiated
varieties. The present case was consistent with
a well differentiated angiosarcoma with char-
acteristic interlacing channels lined by atypi-
cal endothelial cells and expression of
immunohistochemical markers of vascular
differentiation (CD34 and FVIII-RA).9 Often
tumours display both vasoformative and
undifferentiated areas.9 The present case was
noteworthy, in that the entire tumour ap-
peared to be well differentiated. The prolifera-
tion rate was low to moderate compared to the
reported rate for angiosarcoma (>10% in 72%
of cases assessed by Ki-67).10 Mitotic index
has been observed as an independent histo-
logical prognostic marker for cutaneous angi-
osarcoma although other histological makers
remain poorly defined.11 In the present case
low proliferation index and well differentiated
histological features may have contributed to
a better outcome.
The most important clinical prognostic fac-
tor is lesion size, with tumours <5 cm in
diameter having a better prognosis.11 This
underlies the importance of early diagnosis
and appropriate management. The mainstay
of treatment for cutaneous angiosarcoma
includes surgery and/or radiotherapy al-
though, due to the rarity of this condition,
there are no standard guidelines.2 12 As illus-
trated by our case, the primary lesions were
contained within the anterior lamella of the
lids and it was possible to surgically encom-
pass the primary lesions avoiding exentera-
tion and without compromising prognosis. In
such cases, the surgical aim is to resect all
clinically identifiable disease. In areas of
doubt, microscopic control of surgical margins
may have a role in guiding the extent of
resection. Angiosarcomas usually respond to
radiotherapy to some degree and most au-
thors suggest that combined surgery and
radiotherapy offers the best chance for long
term control.2 In one series of 28 head and
neck angiosarcomas, Mark et al12 have re-
ported better survival after 32 months (me-
dian) with combined surgery and radio-
PostScript
therapy compared with surgery alone. In
cases unsuited to surgery, radiotherapy alone
may be considered, however usually only par-
tial responses are achieved.2 12 The role of
chemotherapy remains undefined.
In summary, cutaneous angiosarcoma
should be included in the differential diagno-
sis of vascular or atypical eyelid lesions as
early recognition is critical to optimise out-
come. Classification of the mitotic index and
degree of histopathological differentiation
may be useful criteria in predicting the
biological behaviour. Treatment needs to be
individualised to the patient and in selected
cases complete excision of clinically evident
disease may be appropriate. Adjuvant radio-
therapy is recommended due to the aggressive
natural history of angiosarcoma.
Acknowledgements
The excellent technical assistance of Carmen
Hofmann-Rummelt is acknowleged. The study was
supported in part by the Alexander von Humboldt
Foundation, Bonn, Germany (RMC), The Deutsche
Forschungsgemeinschaft, Bonn, Germany (TH), Al-
lergan Australia in conjunction with The Royal Aus-
tralian and New Zealand College of Ophthalmolo-
gists and Sydney Eye Hospital (RMC).
R M Conway, T Hammer, A Viestenz,
L M Holbach
Department of Ophthalmology and Eye Hospital,
University of Erlangen-Nürnberg, Erlangen,
Germany
R M Conway
Alexander von Humboldt Fellow and Lecturer Save
Sight Institute, University of Sydney, Australia
Corresondence to: R Max. Conway, MD, PhD,
Department of Ophthalmology and Eye Hospital,
University Erlangen-Nürnberg, Schwabachanlage
6, D-91054 Erlangen, Germany;
rmaxconway@hotmail.com
Accepted for publication 8 October 2002
References
1 Holden CA, Spittle MF, Wilson Jones E.
Angiosarcoma of the face and scalp,
prognosis and treatment. Cancer
1987,59:1046–57.
2 Morrison WH, Byers RM, Garden AS, et al.
Cutaneous angiosarcoma of the head and
neck. A therapeutic dilemma. Cancer
1995;76:319–27.
3 Panizzon R, Schneider BV, Schnyder UW.
Rosacea-like angiosarcoma of the face.
Dermatologica 1990;181:252–4.
4 Bray LC, Sullivan TJ, Whitehead K.
Angiosarcoma of the eyelid. Aust NZ J
Ophthalmol 1995;23:69–72.
5 Gündüz K, Shields JA, Shields CL, et al.
Cutaneous angiosarcoma with eyelid
involvement. Am J Ophthalmol
1998;125:870–1.
6 Lapidus CS, Sutula FC, Stadecker MJ, et al.
Angiosarcoma of the eyelid: yellow plaques
causing ptosis. J Am Acad Dermatol
1996;34:308–10.
7 Mehrens C, Anvari L, Grenzebach UH, et al.
Unilateral eyelid swelling as an initial
manifestation of angiosarcoma. Der Hautarzt
2000;51:419–22.
8 Tay YK, Ong BH. Cutaneous angiosarcoma
presenting as recurrent angio-oedema of the
face. Br J Derm 2000;143:1346–8.
9 Oshawa M, Naka N, Tomita Y, et al. Use of
immunohistochemical procedures in
diagnosing angiosarcoma. Evaluation of 98
cases. Cancer 1995;75:2867–74.
10 Meis-Kindblom JM, Kindblom LG.
Angiosarcoma of soft tissue: a study of 80
cases. Am J Surg Pathol 1998;22:683–97.
11 Naka N, Ohsawa M, Tomita Y, et al.
Prognostic factors in angiosarcoma: a
multivariate analysis of 55 cases. J Surg
Oncol 1996;61:170–6.
PostScript
12 Mark RJ, Tran LM, Sercarz J, et al.
Angiosarcoma of the head and neck. The
UCLA experience 1955 through 1990. Arch
Otolaryngol Head and Neck Surg
1993;119:973.
Permanent visual loss in a child
with a rash
Endophthalmitis is an unusual but well
described complication of bacteraemia. Here
we report endophthalmitis arising in the
course of a mild illness resembling chicken-
pox.
Case report
A previously healthy 6 year old white girl
awoke in the early hours feeling hot and com-
plaining of headache. She was given ibupro-
fen and she went back to sleep. Spots
appeared on her limbs later the same day but
they blanched beneath a glass bottle and sev-
eral developed into small blisters. A classmate
was known to have chickenpox. Next day
some new spots appeared on her buttocks,
and some of the older lesions appeared to be
crusting over. She was noticed rubbing her
right eye and later said she felt warm, and
vomited. Next morning she complained of
pain in the right eye and could not open it.
Following assessment by her general prac-
titioner she was referred urgently to an eye
unit.
On examination she appeared well and was
afebrile. The right eye was chemotic, with an
absent red reflex and a large hypopyon (Fig
1). Erythematous maculopapular lesions were
present on her arms, legs, and face.
Investigations included a white cell count
of 8.0 × 109/l (neutrophils 4.0 × 109/l) and C
reactive protein 22 mg/l. Material was col-
lected from the base of the sole remaining
vesicle, on her shin. Ciprofloxacin was com-
menced parenterally at a dose of 100 mg twice
daily (10 mg/kg/day). Examination under
general anaesthesia revealed a panophthalmi-
tis with total corneal abscess and hypopyon.
There was no evidence of a puncture wound to
suggest exogenous infection. Aqueous hu-
mour was aspirated, and intravitreous ami-
kacin and vancomycin administered. Vitrec-
tomy could not be performed because of the
severe anterior segment inflammation.
Electron microscopy, immunofluorescence
staining and polymerase chain reaction (PCR)
testing of the vesicular material was negative
for varicella zoster virus (VZV) and herpes
simplex virus. Neither IgM nor IgG against
Figure 1 Right eye showing
panophthalmitis. There is a diffuse corneal
infiltrate and a purulent exudate in the
anterior chamber leading to the opaque
appearance of the cornea and the absence
of any view of the iris or pupil. The sclera is
exposed in the upper temporal quadrant and
is infiltrated and thinned.
VZV was detected in the blood. Cultures of
blood and aqueous humour were negative but
urgent Gram stain of the aqueous humour
revealed scanty intracellular Gram negative
diplococci. Meningococcus C DNA was de-
tected in the aqueous and in the skin
scraping, but not in blood, by PCR testing.
The patient was apyrexial and remained
clinically well during her hospital admission.
At discharge after 5 further days of parenteral
antibiotics (ceftriaxone) the rash had com-
pletely resolved. In the absence of a positive
culture sensitivities could not be confirmed,
although all the stated antibiotics except van-
comycin are generally effective against
meningococcus. At follow up the eye had
become phthisical, with visual acuity reduced
to perception of light only.
Comment
In this case meningococcal endophthalmitis,
usually seen in association with clinically
apparent septicaemia and meningitis,1 oc-
curred in the setting of a mild illness thought
to be chickenpox. Although the usual hall-
marks of meninogococcal sepsis were absent,
evidence for seeding of the eye in the course of
a transient meningococcaemia was provided
by Gram negative intracellular diplococci in
the aqueous humour and detection of menin-
gococcal DNA in both the aqueous humour
and cutaneous material. Reports of meningo-
coccal endophthalmitis in clinically well, non-
bacteraemic patients are very rare, with just
four published cases in English. The patients
were 13 months,2 15 years,3 17 years,4 and 23
years5 old. Both eldest and youngest had no
skin lesions, but were pyrexial at presentation.
The 15 year old had a low grade fever and a
generalised macular rash misdiagnosed as
scarlet fever. The 17 year old appeared
completely “non-septic” with a 4 day history
of myalgia, arthralgia, sore throat, and tran-
sient fever. A vesicular rash on the lower
extremities appeared vasculitic on histological
examination. In all of these cases only one eye
was involved. Meningococcus type C was iso-
lated from the eye in two cases, type B in a
third, and in the fourth case the type was not
stated. The outcome was bad in each case. In
the three older patients vision was reduced to
the perception of light only, with dense
cataracts forming in two, and pthisis in the 23
year old who underwent vitrectomy. The 13
month old underwent vitrectomy and lensec-
tomy, and the plan at publication was to pro-
vide the patient with a new lens.
Vesicles are not generally regarded as lying
within the spectrum of rashes caused by
meningococcal infection. The acute ocular
complications of varicella infection include
spread of vesicles to the eyelid, papillary con-
junctivitis and, uncommonly, dendritic kerati-
tis, acute retinal necrosis, and optic neuritis.6
Hypopyon, observed in this case, is not a fea-
ture of primary VZV infection.
This report serves to emphasise the broad
clinical spectrum of meningococcal disease,
and highlights the potential for severe seque-
lae despite an otherwise effective host re-
sponse to bacteraemia. Seeding of group C
meningococci resulted in visual loss in this
young patient for whom the UK conjugate
vaccine programme came just a year too late.
P A Berry, J K Dart, E Graham,
M Matheson, E M E MacMahon
St Thomas’s Hospital, London UK
515
Correspondence to: Philip Berry, St Thomas’s
Hospital, London UK; philaberry@hotmail.com
Accepted for publication 10 October 2002
References
1 Steven N, Wood M. The clinical spectrum of
meningococcal disease. In: Cartwright K, ed.
Meningococcal disease. Chichester: John
Wiley, 1995:177–205.
2 Auerbach SB, Leach CT, Bateman BJ, et al.
Meningococcal endophthalmitis without
concomitant septicaemia or meningitis. Paed
Inf Dis J 1989;8:411–13.
3 Brisner JH, Hess JB. Meningococcal
endophthalmitis without meningitis. Can J
Opthalmol 1981;16:100–1.
4 Sleep T, Graham M. Meningoccal
endophthalmitis in a well patient [letter]. J
Opthalmol 1997; 81:1016–20.
5 Kearns AM, Sprott MS. Endophthalmitis
caused by Neisseria meningitidis. J Infect
1991;22:299–300.
6 Ritterband DC, Friedberg DN. Virus infection
of the eye. Rev Med Virol 1998;8:187–201.
MAILBOX
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Those excrescences on
Descemet’s membrane
We read with interest the recent report,
“Screening human donor corneas during
organ culture for the presence of guttae.”1
Even the title made us question whether we
were the only ones still using the original
name for the condition cornea guttata. A quick
perusal of Medline reveals a trend away from
guttata, and towards guttae (Fig 1). This trend
may be even more pronounced, as the
literature tends to be conservative. The next
generation of cornea specialists, or at least the
small sample that goes through our training
programme, tends to fall into three categories:
those who say guttae, those who correct others
for saying guttata, and those who try their best
not to mention the term for fear they’re saying
the wrong thing. Many of our faculty have
now entered this last group.
When asked what is so bad about guttata,
some insist that it is not the plural for gutta,
the Latin word for drop. We’re not sure that
anyone ever said it was. More erudite dissent-
ers recognise that not only is guttata not a
plural, it is not even a noun—it’s an adjective.
The closest English counterpart to guttata is
probably guttate, drop-like, or speckled,2 so it’s
incorrect to use guttata alone as a noun, or so
the thinking goes. Some have chosen to use
cornea guttata when referring to the condi-
tion, and guttae when referring to the excres-
cences themselves.1
www.bjophthalmol.com
516
Figure 1 References to guttata and guttae
over the past 40 years in Medline. Some
articles use both terms.1
“Guttata were present” may well have
grated the ears of an ancient Roman, since
guttata is feminine singular, and Romans
liked their subjects to agree with their verbs in
number as much as the rest of us. However, it
is not so clear that “Flavia’s guttata is impres-
sive” would be at all offensive, particularly
since blonde Flavia herself was named after
an adjective, as were Augustus and, some say,
even Caesar himself. In fact, early Roman
grammarians barely distinguished between
adjectives and nouns, using them inter-
changeably any chance they could.3 The
learned still do, at least when they refer to
themselves as the learned. Those particularly
offended by using an adjective substantively
(as a noun) should be careful not to slip up
with words like cornea. Both cornea and con-
junctiva started out their ophthalmological
careers as adjectives, modifying tunica (coat,
feminine singular), which is why cornea and,
in turn, guttata are feminine singular in the
first place.
What then should we call those excres-
cences on Descemet’s membrane? Well, if you
think they are drops, then by all means call
them drops, although you probably don’t need
to invent yet another term from a dead
language. Vogt called them “tropfige” (drop-
like) Prominenzen der Hornhautrückfläche
before he coined cornea guttata (his quotes,
not ours).4 If, like Dr Vogt, you want to convey
that the cornea looks speckled with drop-like
excrescences, then you could use the substan-
tive adjective guttata as a singular noun, or
the English guttate as an adjective. You could
even use guttata as a plural noun—if criticised
just say you were referring to the many drop-
like excrescentia (neuter plural), not a single
speckled tunica cornea (feminine singular). A
resident on our service recently wrote in a
chart note guttatae (feminine plural). We cer-
tainly found no fault. In fact, it may be a rea-
sonable compromise for those who have
sworn never to use guttata. We find no
reference to it yet in Medline.
T Lietman, J Lee, S Costanza
FI Proctor Foundation and the Department of
Ophthalmology, University of California, San
Francisco, USA
Correspondence to: Thomas Lietman, MD, WHO
Collaborating Center, Department of
Ophthalmology, FI Proctor Foundation, University of
California San Francisco, 95 Kirkham Street, Room
307, San Francisco, CA 94143–0944, USA;
tml@itsa.ucsf.edu
References
1 Borderie V, Sabolic V, Touzeau O, et al.
Screening human donor corneas during organ
culture for the presence of guttae. Br J
Ophthalmol 2001;85:272–6.
2 Glare PG. Oxford Latin dictionary. Oxford:
Oxford University Press, 1983.
www.bjophthalmol.com
3 Humez A, Humez N D. Latin for people,
Latina pro populo. Boston: Little, Brown,
1976.
4 Vogt A. Lehrbuch und Atlas
Spaltlampenmikroskopie des Lebenden Auges.
Berlin: Verlag von Julius Springer, 1930.
Acanthamoeba keratitis
We read with interest the report by Radford et
al on the incidence of acanthamoeba keratitis
and the suggested relation with hardness of
water.1 However, we disagree with their
findings and believe their study is flawed for
two reasons.
Firstly, the incidence of acanthamoeba
keratitis has been underestimated by approxi-
mately 33% since their monthly questionnaire
reporting system yielded an average return of
only 67%. A high non-response rate over 30%
is considered unreliable for making inferences
for the whole population.2 Furthermore, the
authors estimated the “incidence” rate using
the “complete” cases, which is a biased
sample. Even then, if the non-response rates
were below 30% in this study, the authors did
not provide the precision of the “incident”
rate and attempt to apply a statistical weight-
ing approach to remove or reduce non-
response bias.
Of 179 positive reports collected, only 106
(59%) met their case definition criteria. This
further reduced their number of cases but will
have contributed to their diagnostic accuracy.
A further five cases were excluded as non-UK
residents but where did they acquire their
infection—in the United Kingdom or over-
seas?
The incidence figures calculated for Eng-
land and Wales for 1997-8 and 1998-9 of 0.21
and 0.18 per 10 000 contact lens wearers
(CLW) are thus an underestimate of the
expected true infection rate. The expected
true figure needs to be increased by a factor of
1.493 (100/67) to account for the missed cases
with the low reporting rate used of 67%. The
English figures then become 0.31 (0.21 ×
1.493) and 0.27 (0.18 × 1.493) per 10 000 CLW
respectively, of whom the majority (88%)
wore soft hydrogel lenses.
These latter (corrected) figures for England
and Wales are remarkably similar to the true
figure estimated at the same time in a 17
month prospective cohort study in Hong
Kong3 of 0.33 per 10 000 “soft” hydrogel CLW.
Reporting “questionnaire” studies of infec-
tious disease, albeit prospective and of new
cases only, usually underestimate the true
incidence figure by at least twofold and that
used by the authors (BOSU) is no exception.
Incidence figures for rare diseases are best
estimated by performing a prospective cohort
study, in which all cases occurring within a
community of two million to three million
people over a defined period (often a year or
more) are examined and tested in several
referral hospitals according to defined criteria
in advance.
Secondly, the authors suggest that there is a
relation between water hardness and acquisi-
tion of acanthamoeba keratitis, on the basis of
correlation of their questionnaire reports with
water quality. They suggest a higher risk in
areas of hard water but such findings are not
supported by our results. In the west of Scot-
land, where there is very soft water (mean
value for calcium carbonate 15.22 mg/l), we
recorded the highest incidence ever of acan-
thamoeba keratitis of 1.49 per 10 000 “soft”
hydrogel CLW in 1994-5 in a prospective
cohort study.4 In Hong Kong,3 with an
incidence of 0.33 per 10 000 CLW, the water is
also soft (mean value for calcium carbonate
40 mg/l).
PostScript
We believe that the suggested correlation by
the authors of acanthamoeba keratitis with
water hardness is spurious and demonstrates
the danger of projecting “correlation” to
“causation” within their study. The authors do
not have definitive evidence to support their
case because of sampling bias that results in
part from evaluating associations between
risk factors and a disease well after the risk
exposure first occurs. During that time inter-
val, cases of short duration or cases mild in
severity or asymptomatic, and cases in which
the presence of disease alters or entirely
removes the exposure, are missed.5
Water “hardness” remains unproved as a
risk factor for acanthamoeba keratitis being
no more than an observational quirk. While
we have had good molecular based evidence
for the association of acanthamoeba keratitis
with home tap water,6 recent work using 18S
rDNA typing and tracking has shown that
there can be different subtypes of keratitis
causing strains of Acanthamoeba spp (T4)
between the tap water in the home and the
cornea, making the epidemiology more
involved.7 8
In conclusion, the incidence of acan-
thamoeba keratitis reported for England and
Wales by the authors is an underestimate of
the true figure by at least 33%. The finding
that the reporting of cases was “static” is of
concern as it means that there is still need to
reinforce ways of preventing this infection.9–11
While considerably less frequent since the
introduction of multipurpose solutions 7
years ago,12 the infection remains a problem to
be avoided by CL wearers as predicted by one
of us 8 years ago.13 If good hygiene is practised,
however, with monthly changing of storage
cases, avoidance of tap water, and use only of
sterile multipurpose solutions or overnight
storage in hydrogen peroxide, then the risk of
infection becomes negligible. Use of true daily
disposable lens wear has also been virtually
free of acanthamoeba infection.
D V Seal
Applied Vision Research Centre, City University,
Northampton Square, London EC1V 0HB, UK
T K Beattie, A Tomlinson
Department of Vision Sciences, Glasgow
Caledonian University, Cowcaddens Road,
Glasgow G4 0BA, UK
D Fan, E Wong
Departments of Ophthalmology and Centre for
Clinical Trials and Epidemiological Research
(School of Public Health), Chinese University of
Hong Kong, Prince of Wales Hospital, Shatin, NT,
Hong Kong
Correspondence to: David Seal;
100756.3112@compuserve.com
References
1 Radford CF, Minassian DC, Dart JKG.
Acanthamoeba keratitis in England and
Wales: incidence, outcome and risk factors.
Br J Ophthalmol 2002;86:536–42.
2 Gail MH, Benichou J. Encyclopedia of
epidemiologic methods. New York: John
Wiley,.2000.
3 Lam D, Houang E, Lyon D, et al. Incidence
and risk factors for microbial keratitis in Hong
Kong: comparison with Europe and North
America. Eye 2002;16:608–18.
4 Seal DV, Kirkness CM, Bennett HGB, et al.
Population-based cohort study of microbial
keratitis in Scotland: incidence and features.
Contact Lens Ant Eye 1999;22:49–57.
5 Sackett DL. Bias in analytical research. J
Chronic Disease 1979;32:51–63.
6 Ledee DR, Hay J, Byers TJ, et al.
Acanthamoeba griffini: molecular
characterisation of a new corneal pathogen.
Invest Ophthalmol Vis Sci 1996;37:544–50.
PostScript
7 Schroeder JM, Booton GC, Hay J, et al. Use
of subgenic 18s ribosomal DNA PCR and
sequencing for genus and genotype
identification of acanthamoebae from humans
with keratitis and from sewage sludge. J Clin
Microbiol 2001;39:1903–11.
8 Booton GC, Kelly DJ, Chu Y-W, et al. 18S
Ribosomal DNA typing and tracking of
Acanthamoeba sp from corneal scrape
specimens, contact lenses, lens cases and
home water supplies of acanthamoeba
keratitis patients in Hong Kong. J Clin
Microbiol 2002;40:1621–5.
9 Fan D, Lam D, Houang E, et al. Health belief
and health practice in contact lens wear—a
dichotomy? CLAO J 2002;28:36–9.
10 Houang E, Lam D, Fan D, et al. Microbial
keratitis in Hong Kong—relationship to
climate, environment and contact lens
disinfection. Trans Roy Soc Trop Med Hyg
2001;95:361–7.
11 Seal DV, Kirkness CM, Bennett HGB, et al.
Acanthamoeba keratitis in Scotland: risk
factors for contact lens wearers. Contact Lens
Ant Eye 1999;22:58–68.
12 Stevenson RWW, Seal DV. Has the
introduction of multi-purpose solutions
contributed to a reduced incidence of
Acanthamoeba keratitis in contact lens
wearers? Contact Lens Ant Eye
1998;21:89–92.
13 Seal DV. Acanthamoeba keratitis. A problem
for contact lens users that is here to stay. BMJ
1994;308:1116–17.
Surgery for glaucoma
We would like to thank Drs Mermoud and
Ravinet for their online comments on the
editorial1 and also for their comments on
O’Brart’s article (eletter posted on www.
bjophthalmol.com 5 Aug 2002).2
We are delighted that there is agreement
that subconjunctival drainage is an important
component of non-penetrating filtering sur-
gery. Blebs, albeit diffuse, are clearly visible
both in cases of deep sclerectomy, and in many
cases of viscocanalostomy. However, although
Ravinet and Mermoud and ourselves are cer-
tain that this is an important component for
drainage in non-penetrating filtration sur-
gery, it is important to clarify this point.
Although we are certain of the importance,
there is still controversy over the relative
importance of subconjunctival drainage ver-
sus other routes such as trans-scleral or via
Schlemm’s canal in the case of viscocanalos-
tomy.
On the second point, although Mermoud
and Ravinet use antimetabolites before cut-
ting into the sclera, they do not apply antime-
tabolites including mitomycin into the deep
scleral bed. Again, although they do not do
this, we know that deep application has been
used in other centres. Clearly, the risk of
intraocular entry is higher in these situations,
and that was the reason for making this com-
ment in the editorial, to warn people of the
possible dangers of applying mitomycin after
a deep scleral dissection.
Regarding the question of additional hypo-
tony after macroperforation, clearly the team
are very experienced in doing this surgery.
However, from canvassing personal opinion
from individuals who are doing this surgery,
who are less experienced than them (like the
majority of surgeons doing non-penetrating
surgery), and also from reports in the
literature including randomised prospective
trials, macroperforation is associated with
early hypotony.3 4 This may well be exacer-
bated if intraoperative antimetabolites, par-
ticularly mitomycin, have been used.
With regard to the comments on the paper
by O’Brart et al, we have forwarded the
contents of the letter on to the authors and
they have replied separately to the comments.
In conclusion, we are pleased that the arti-
cle and editorial have led to further healthy
discussion. Mermoud and his colleagues are
to be commended for the work they continue
to do in non-penetrating filtration surgery.
However, as we said in our editorial, current
studies do reveal that none of the current
operations for glaucoma are totally ideal yet,
and further research, particularly on surgical
methods and wound healing control, is
needed so that optimal long term pressure
control can be achieved for all our patients
with a minimum of complications.
P T Khaw, A P Wells, K S Lim
Moorfields Eye Hospital and the Institute of
Ophthalmology, Bath Street, London EC1V 9EL, UK
Correspondence to: Peng T Khaw;
p.khaw@ucl.ac.uk
References
1 Khaw PT, Wells AP, Lim KS. Surgery in the
21st century. Br J Ophthalmol
2002;86:710–11.
2 O’Brart DPS, Rowlands E, Islam N, et al. A
randomised, prospective study comparing
trabeculectomy augmented with
antimetabolites with a viscocanalostomy
technique for the management of open angle
glaucoma uncontrolled by medical therapy. Br
J Ophthalmol 2002;86:748–54.
3 Chiselita D. Non-penetrating deep
sclerectomy versus trabeculectomy in primary
open angle glaucoma surgery. Eye
2001;15:197–201.
4 El Sayyad F, Helal M, El Kholify H, et al.
Nonpenetrating deep sclerectomy versus
trabeculectomy in bilateral primary open
angle glaucoma. Ophthalmology
2000;107:1671–4.
A randomised controlled trial of
written information
Newsham’s effort to inform parents of chil-
dren with amblyopia about occlusion therapy
is laudable but incomplete.1 Ethical considera-
tions of informed consent require full disclo-
sure of all aspects of the proposed treatment.
The following points might be considered for
inclusion.
(1) Occlusion therapy has never been
scientifically validated with a randomised,
controlled study.
(2) The dose/response relation has never
been defined. Flynn et al stated that “Success
was not related to the duration of occlusion
therapy, type of occlusion used . . .”2 The vari-
ety of treatment protocols accentuates an-
other dilemma “owing to our paucity of
knowledge on the dose-effect relation—a
situation one finds hard to imagine for any
comparably established therapy outside oph-
thalmology. In other words we have no
understanding of the dose-effect relation of
occlusion in amblyopia therapy.”3
(3) The application of “greater levels of
occlusion being prescribed for more severe
amblyopia”1 is compromised by the observa-
tion “that success was related to . . . the depth
of visual loss before treatment . . .”4
(4) The benefits of treatment are likely to
deteriorate following cessation of patching.5
(5) Visual acuity improves as children
become more mature, literate, and familiar
with vision testing protocols.6 This is also true
for amblyopic eyes. In amblyopic children
between 3 and 7 years old without treatment
visual acuity was shown to consistently
improve in each older age group.7
(6) Both the occluded and the amblyopic
eyes improve at the same rate during
treatment.8
517
(7) Success in amblyopia treatment is usu-
ally defined as improvement by a minimum of
three lines.9 Many of the successfully treated
patients, by that criterion, will still not have
normal vision at the end of presumably
successful treatment. One quarter of treated
patients with initial acuity better than 20/100
do not even achieve these limited goals.9
Therefore, the comments about achieving
normal vision may raise expectations that will
not be achieved.
(8) Occlusion therapy does have potential
adverse effects beyond disruption of family
and social life10 and interference with
education.11
(9) Despite decades of occlusion therapy
the prevalence of amblyopia in the adult
population is similar to that of the school age
population.12 Moreover, “the prevalence of
unilateral amblyopia was not found to be sta-
tistically different by age group.”13 This
suggests that long term benefits of conven-
tional therapy are not demonstrated in demo-
graphic studies.
Patients and their families should be
provided with comprehensive information
concerning proposed treatments. Physicians
are obliged to make this information accurate
and inclusive.2
P Lempert
Cornell University School of Veterinary Medicine,
Ithaca, NY 14850, USA; eyechartplus@aol.com
References
1 Newsham D. A randomised controlled trial
of written information: the effect on parental
non-concordance with occlusion therapy. Br J
Ophthalmol 2002;86:787–91.
2 Flynn JT, Schiffman J, Feuer W, et al. The
therapy of amblyopia: an analysis of the
results of amblyopia therapy utilizing the
pooled data of published studies. Trans Am
Ophthalmol Soc 1998;96:431–50; discussion
450–3.
3 Simonsz HJ, Polling JR, Voorn R, et al.
Electronic monitoring of treatment compliance
in patching for amblyopia. Strabismus
1999;7:113–23.
4 Beardsell R, Clarke S, Hill M. Outcome of
occlusion treatment for amblyopia. J Pediatr
Ophthalmol Strabismus 1999;36:19–24.
5 Von Noorden GK, Attiah F Alternating
penalization in the prevention of amblyopia
recurrence. Am J Ophthalmol
1986;102:473–5.
6 Robinson BE, Oladeji MM, Bobier WR.
Visual acuity assessment in preschool children
in Oxford County. ARVO 2000 Abstract No
4955.
7 The Pediatric Eye Disease Investigator
Group. The clinical profile of moderate
amblyopia in children younger than 7 years.
Arch Ophthalmol 2002;120:281–7.
8 Dorey SE, Adams GG, Lee JP, et al. Intensive
occlusion therapy for amblyopia. Br J
Ophthalmol 2001;85:310–13.
9 The Pediatric Eye Disease Investigator
Group. A randomized trial of atropine vs
patching for treatment of moderate amblyopia
in children. Arch Ophthalmol
2002;120:268–78.
10 Snowdon SL, Stewart-Brown SL. Amblyopia
and disability: a qualitative study. Oxford:
Health Services Research Unit University of
Oxford.
11 Yang LL, Lambert SR. Reappraisal of
occlusion therapy for severe structural
abnormalities of the optic disc and macula. J
Pediatr Ophthalmol Strabismus
1995;32:37–41.
12 Buch H, Vinding T, La Cour M, et al. The
prevalence and causes of bilateral and
unilateral blindness in an elderly urban Danish
population. The Copenhagen City Eye Study
Acta Ophthalmol Scand 2001;79:441–9.
13 Brown SA, Weih LM, Fu CL, et al. Prevalence
of amblyopia and associated refractive errors
www.bjophthalmol.com
518
in an adult population in Victoria, Australia.
Ophthalmic Epidemiol 2000;7:249–58.
Ocular and financial health
We read with interest the article by
Khandekar et al detailing the results of the
1996–7 Omani Eye Study.1 They found a
prevalence of blindness of 16.8% in those 60+
years of age, and comment that the popula-
tion of this age group in Oman is predicted to
double by the year 2020. This has serious
implications for planning the provision of
health care and specifically for eye care
services.
This observation is true for most countries;
the global population for the over 65 age
group is projected to increase from approxi-
mately 400 million to 800 million people by
the year 2020, contrasting with the under 5
years of age population, which is estimated to
see a 6% growth in the same period.2
“Vision 2020—the right to sight” was
launched by the WHO and IAPB in 1999, aim-
ing for the elimination of avoidable blindness
by the year 2020. In 1995 the estimate of glo-
bal blindness (<3/60 better eye) was 44
million, and this is projected to rise to 76 mil-
lion by 2020 if there is no change in current
trends.3 Vision 2020 prioritises five diseases
for global attention—cataract, refractive er-
rors, trachoma, onchocerciasis, and vitamin A
deficiency. Action against diabetic retinopathy
and glaucoma is also deemed important in
countries where ocular infections have been
controlled. Vision 2020 contends that the cur-
rent increase in blindness, estimated at 1–2
million people per year, can be reversed if
human and financial resources are targeted at
these priority diseases in the countries with
the highest prevalence and number of blind
people. It is estimated that the result of a suc-
cessful programme, achievable at a cost of $2
billion, will be 429 million blind person years
avoided over the next 20 years, and a total
saving in excess of $100 billion, by avoidance
of lost productivity.3 This would make the
effective implementation Vision 2020 not only
ethically important but also a cost effective
strategy for poverty alleviation.
J C Buchan, C Gilbert
London School of Hygiene and Tropical Medicine,
Keppel Street, London WC1E 7HT, UK
Correspondence to: John Buchan;
john.buchan@lshtm.ac.uk
References
1 Khandekar R, Mohammed AJ, Negrel AD, et
al. The prevalence and causes of blindness in
the Sultanate of Oman: the Oman Eye Study
(OES). Br J Ophthalmol 2002;86:957–62.
2 www.census.gov/ipc/prod/wp98/wp98.pdf
3 Frick KD, Foster A. The magnitude and cost
of global blindness: an increasing problem
that can be alleviated. Am J Ophthalmol (in
press).
BOOK REVIEWS
Ocular Pathology, 5th ed.
Myron Yanoff, Ben S Fine. Pp 700; 180. St
Louis: Mosby, 2002. ISBN 0323014038.
The fifth edition of this well established ocular
pathology text is excellent. The presentation,
the photographs, the histopathology, and
micrograph sections provide the reader with
an array of outstanding examples of ocular
www.bjophthalmol.com
pathology. The text is easy to read, agreeably
not always in depth, but gives enough
information or direction in references for fur-
ther reading. This is being hypercritical of an
enjoyable text and there is no more to say
except that every unit and library should
update their edition.
A Dick
The History of Strabismology:
Hirschberg History of
Ophthalmology: The
Monographs.
von Noorden GK, ed. US$145. Belgium:
Wayenborgh, 2002.
For those who specialise in the treatment of
ocular motility disorders, it seems at times as
though time has stood still. Compared with
the technical and surgical innovations that
have characterised the modern history of the
treatment of cornea and retinal diseases, stra-
bismologists have continued to use tech-
niques and instruments devised in the mid-
19th century. However, after reading von
Noorden’s The History of Strabismology, it be-
comes clear that the history of our specialty
has been characterised by physicians and sci-
entists possessing remarkable creativity and
intellect striving to understand how the eyes
move and the brain sees.
The History of Strabismology is the ninth
volume of The History of Ophthalmology, a
planned 21 volume series of monographs
intending to comprehensively review the his-
tory of ophthalmology from ancient times to
the current day. The author has chosen to
review the history of strabismology not with a
traditional “art through the ages” chronologi-
cal approach, but rather from a geocentric
approach. By enlisting prominent strabis-
mologists from around the world as contrib-
uting authors, von Noorden allows the reader
to discover how the discipline and practice of
strabismology developed and evolved not just
in Europe and the United States, but in coun-
tries and regions such as Mexico, Japan,
South America, and Australia. In thoroughly
researched and referenced chapters, the au-
thors describe, time and again, how an
ophthalmologist visiting Europe learns a
technique, returns to his or her country,
refines the technique and applies it to the
patient population unique to his or her coun-
try. We learn of scoundrels and rogues who
foist themselves on the public as miracle
workers, only to be publicly exposed and dis-
credited by ethical ophthalmologists of the
day (lessons we could stand to relearn now
again in the 21st century). We learn that aes-
thetic ideals are relative to time and place;
such as the fact that in pre-Columbian times a
slight degree of esotropia was found to be
attractive and convergence was stimulated in
infants by attaching a ball of beeswax to the
child’s hair to be left dangling between the
eyes (vision therapy at its birth).
An often neglected but historically impor-
tant discipline within the field of strabismus
is the practice of orthoptics. In a carefully
researched and beautifully illustrated chapter,
section author Roper-Hall outlines the origins
of the specialty and introduces us to the
pioneering women and men who selflessly
served, taught, and discovered in the clinics of
the more famous titans of strabismology.
Throughout the book, von Noorden takes
pains to illuminate the lives and contributions
of both major and minor players in the history
PostScript
of strabismus. The reader cannot help but see
how the advancement of science in a disci-
pline is dependent upon the close collabora-
tion that takes place between mentor and
student, doctor and patient, and clinician and
scientist.
As with all the monographs in this series,
the volume contains extensive illustrations,
photographs and reproductions. Portraits and
photographs of innovators in the field of stra-
bismology flesh out the names we associate
with instruments and techniques. Each sec-
tion author provides numerous references of
seminal papers on strabismus published from
the corners of the globe. It is refreshing to see
a book on history recognise the contributions
from those outside the traditional medical
centres of Europe/United Kingdom and the
United States.
This book will be a valuable reference for all
those who specialise in the area of strabismus
and those interested in the history of ophthal-
mology. The illustrations and historical refer-
ences will greatly enhance the quality of
lectures on the topic of strabismus. Knowl-
edge of the origins of critical thought and
technical innovation concerning the aetiology
and treatment of strabismus will stimulate
further interest in today’s students of oph-
thalmology. Finally, the knowledge that the
pioneers of ophthalmology—von Graefe,
Muller, Donders, and Helmholtz—placed the
study and treatment of strabismus at an equal
level of importance as the treatment of
diseases of the lens, cornea, and retina serves
notice to contemporary vision scientists and
ophthalmologists not to neglect this most
challenging discipline of ophthalmology—
strabismus.
D R Fredrick
San Francisco
NOTICES
Role of optometry in Vision 2000
The latest issue of Community Eye Health (No
43) discusses the mobilisation of optometry to
deal with uncorrected refractive error, which
is now a major cause of functional blindness.
For further information please contact: Jour-
nal of Community Eye Health, International
Centre for Eye Health, Institute of Ophthal-
mology, 11–43 Bath Street, London EC1V 9EL,
UK (tel: +44 (0)20 7608 6910; fax: +44 (0)20
7250 3207; email: eyeresource@ucl.ac.uk;
web site: www.jceh.co.uk). Annual subscrip-
tion (4 issues) UK£25/US$40. Free to workers
in developing countries.
International Centre for Eye
Health
The International Centre for Eye Health has
published a new edition of the Standard List of
Medicines, Equipment, Instruments and Optical
Supplies (2001) for eye care services in
developing countries. It is compiled by the
Task Force of the International Agency for the
Prevention of Blindness. Further details: Sue
Stevens, International Centre for Eye Health,
11–43 Bath Street, London EC1V 9EL,
UK (tel: +44 (0)20 7608 6910; email:
eyeresource@ucl.ac.uk).
Second Sight
Second Sight, a UK based charity whose aims
are to eliminate the backlog of cataract blind
in India by the year 2020 and to establish
PostScript
strong links between Indian and British oph-
thalmologists, is regularly sending volunteer
surgeons to India. Details can be found at the
charity web site (www.secondsight.org.uk) or
by contacting Dr Lucy Mathen (lucymathen@
yahoo.com).
SPecific Eye ConditionS (SPECS)
SPecific Eye ConditionS (SPECS) is a not for
profit organisation which acts as an umbrella
organisation for support groups of any condi-
tions or syndrome with an integral eye disor-
der. SPECS represents over fifty different
organisations related to eye disorders ranging
from conditions that are relatively common to
very rare syndromes. We also include groups
who offer support of a more general nature to
visually impaired and blind people. Support
groups meet regularly in the Boardroom at
Moorfields Eye Hospital to offer support to
each other, share experiences and explore new
ways of working together. The web site
www.eyeconditions.org.uk acts as a portal
giving direct access to support groups own
sites. The SPECS web page is a valuable
resource for professionals and may also be of
interest to people with a visual impairment or
who are blind. For further details about
SPECS contact: Kay Parkinson, SPECS Devel-
opment Officer (tel: +44 (0)1803 524238;
email: k@eyeconditions.org.uk;web site:
www.eyeconditions.org.uk).
The British Retinitis Pigmentosa
Society
The British Retinitis Pigmentosa Society
(BRPS) was formed in 1975 to bring together
people with retinitis pigmentosa and their
families. The principle aims of BRPS are to
raise funds to support the programme of
medical research into an eventual cure for this
hereditary disease, and through the BRPS
welfare service, help members and their fami-
lies copy with the everyday concerns caused
by retinitis pigmentosa. Part of the welfare
service is the telephone help line (+44
(0)1280 860 363), which is a useful resource
for any queries or worries relating to the
problems retinitis pigmentosa can bring. This
service is especially valuable for those recently
diagnosed with retinitis pigmentosa, and all
calls are taken in the strictest confidence.
Many people with retinitis pigmentosa have
found the Society helpful, providing encour-
agement, and support through the Help line,
the welfare network and the BRPS branches
throughout the UK. (tel: +44 (0)1280 821
334; email: lynda@brps.demon.co.uk; web
site: www.brps.demon.co.uk)
Surgical Eye Expeditions
International
Volunteer ophthalmologists in active surgical
practice are needed to participate in short
term, sight restoring eye surgery clinics
around the world. Contact: Harry S Brown,
Surgical Eye Expeditions International, 27
East De La Guerra, C-2, Santa Barbara, CA
93101-9858, USA (tel: +805 963 3303;
fax: +805 965 3564; email:
hsbrown.md@cox.net or seeintl@seeintl.org;
web site: www. seeintl.org).
16th Annual Meeting of German
Ophthalmic Surgeons
The 16th Annual Meeting of German Oph-
thalmic Surgeons will be held 8–11 May 2003
in Nürnberg, Germany, Messezentrum. Or-
ganised by the Professional Association of
German Ophthalmologists Ophthalmic Sur-
gery Group the conference will cover cataract
surgery, refractive surgery, glaucoma surgery,
vitreoretinal surgery, corneal surgery, eye sur-
gery in developing countries, and orbita,
lacrimal and lid surgery. Further details: MCN
Medisinische Congress organisation Nürn-
berg AG, Zerzabelshofstr 29, 90478 Nürnberg,
Germany (tel:+49 911 3931621; fax: +49 911
3931620; email: doc@mcnag.info; web site:
www.doc-nuernberg.de).
3rd British Oculoplastic Surgery
Society Meeting
The 3rd British Oculoplastic Surgery Society
Meeting will be held 18–19 May 2003 in
Birmingham, UK. For further details please
contact the Secretary of the British Oculoplas-
tic Surgery Society Jane Olver (tel: +44
(0)121 424 5464; fax: +44 (0)121 424 4464;
email: MartiDi@heartsol.wmids.nhs.uk; web
site: www.bopss.org).
13th Meeting of the EASD Eye
Complication Study Group
The 13th Meeting of the EASD Eye Complica-
tion Study Group will be held on the 23–25
May 2003, in Prague, Czech Republic. The sci-
entific programme includes keynote lectures
from Professor John H Fuller (UK) on The
epidemiology of diabetic retinopathy; Dr P
Martin van Hagen (The Netherlands) on
Growth factors and diabetic retinopathy; Pro-
fessor Terezie Pelikanova (Czech Republic) on
Pathophysiology of diabetic microvascular
complications; Dr Tomas Sosna (Czech Re-
public) on Risk and protective factors of
diabetic retinopathy.
519
Further details: Ortopedicke Centrum,
s.r.o., Strekovské nabrezi 51, 400 03 Usti nad
Labem, Czech Republic (tel: +420 47 521
6588; fax: +420 47 533 40 77; email:
ortcentrum-ul@volnv.cz; web site:
www.ortopedicke-centrum.cz).
IOIS—VIIth International
Symposium on Ocular
Inflammation
The IOIS—VIIth International Symposium on
Ocular Inflammation will be held 25–30 May
2003, in Padova, Italy.
Programme and Organising Committee:
Prof. Antonio Secchi, Chairman of IOIS
and Convenor of Congress (email:
ag.secchi@unipd.it). Further details: Con-
gress Secretariat, Meet and Work, Mario Sbal-
chiero (tel: +39 049 860 1818; fax: +39 049
860 2389; email: info@iois-2003.com; web
site: www.meetandwork.com).
XXXII Retinal Detachment Course
The XXXII Retinal Detachment Course with
International Faculty and Case Presentations
will be held 5–6 June 2003, in Beijing, China.
The Congress language is English with simul-
taneous translation in Chinese. Further de-
tails: Scientific programme: Prof Ingrid Kreis-
sig, University of Tuebingen, Schleichstr. 12,
Breuningerbau, 72076 Tuebingen, Germany
(tel: +49 7071 295209; email:
ingrid.kreissig@med.uni-tuebingen.de).
Local organisation: Ningli Wang, Liang Xu,
Wu Liu. Congress office: Wu Liu, Beijing Tong
Ren Eye Center, Beijing 100730 China (email:
wuliubj@yahoo.com).
Detachment Course with
international faculty on: Retinal
and Vitreous Surgery with Case
Presentations preceding the
Annual Meeting of Iranian
Society of Ophthalmology
The detachment course with international
faculty on: Retinal and Vitreous Surgery with
Case Presentations preceding Annual Meeting
of Iranian Society of Ophthalmology will be
held on 29–30 November 2003 and 1–4
December 2003 respectively, at the Razi
Conference Center, Hemmat Hyw, Tehran,
Iran. Further details: Scientific programme:
Prof Ingrid Kreissig, University of Tuebingen,
Schleichstr. 12, Breuningerbau, 72076
Tuebingen, Germany (tel: +49 7071 295209;
email:ingrid.kreissig@med.uni-tuebingen.de).
Local organisation: Dr Arman Masheyekhi, Dr
Siamak Moradian, Dept of Ophthalmology,
Labbanfinejad Medical Center, Pasdaran Ave,
Boostan 9, Tehran, 16666, Iran (fax: +98 21 254
9039; email: labbafi@hotmail.com).
www.bjophthalmol.com